Manuscripts
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SUBMITTED TO THE JOURNAL AS ON 25 DECEMBER 2011
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journal.
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Aleukemic Myeloid Sarcoma Presenting as Compressive Myelopathy
Myeloid sarcoma, earlier called granulocytic sarcoma or chloroma is a localized extramedullary tumor composed of malignant cells of the myeloid cell line. It occurs most frequently in association with acute myelogenous leukemia or as extramedullary relapse in AML but can present primarily without frank acute leukemia, frequently involving spinal cord, as in a case summarized below or other sites of central or peripheral nervous system. Back to Top -
Adrenocortical Tumors in Children: A Kuwait Experience
Introduction: Adrenocortical tumors (ACT) are rare tumors of childhood. The majority of these tumors are hormone-producing and cause virilization and Cushing’s syndrome or feminization. Methodology: The authors describe 6 cases of adrenal cortical tumors treated in the Kuwait Cancer Center; which were presented over a period of 20 years (1989-2009). Results: The mean age was 5.5 years (range 15 months – 12 years). All had signs of virilization. One child had hypertension, while 2 had a metastatic disease at presentation. The diagnosis was made by clinical signs and symptoms, high levels of relevant adrenal hormones and imaging. Two children were not fit for surgery; one was too sick for any treatment and died shortly after diagnosis, while the other died after receiving one cycle of palliative chemotherapy. Four patients underwent complete surgical resection and achieved complete remission, three of whom later had recurrence (distant in one and local in two patients) and succumbed due to progressive disease. Mitotane was used in two children. Only one patient is currently surviving and well nearly 13 years after her surgery. In our series, the long term outcome of children with adrenocortical tumors was very poor. Conclusions: Virilization is an important clue to the diagnosis of ACT. Early diagnosis and complete surgical resection are important for survival. Metastasis at presentation or as recurrence carries very dismal prognosis. Back to Top -
Second Primary Tumour in the Intraoral Skin Pedicle of Pectoralis Major Myocutaneous Flap Seven years after Reconstruction
Secondary primary tumor developing in the skin paddle of a myocutaneous flap is a rare event. We are reporting a case of squamous cell carcinoma arising in the skin of pectoralis major myocutaneous flap (PMMC) used for the reconstruction of oral cavity. Back to Top -
Gum hypertrophy in a patient of multiple leiomyomata cutis receiving high dose nifedepine – Report of a case and review of literature
Gum hypertrophy has been reported to occur as a side effect of long term use of calcium channel blockers, cyclosporine, tacrolimus, phenytoin, etc. Here we report a case of gum hypertrophy in a patient of (painful) cutaneous leiomyomata on prolonged and high dose nifedipine. Back to Top -
Mixed germ cell tumor of ovary and Clitoromegaly in Swyer's Syndrome: A case report
Swyer syndrome is a type of pure gonadal dysgenesis correlating with 46XY karyotype, primary amenorrhea, and female internal and external genitalia. It reveals a testicular differentiation abnormality. A 16 year old girl admitted to our center with primary amenorrhea and abdominal mass. Inspite of the absence of normal testis, clitoromegaly was noticed. Peripheral blood karyotype analysis showed 46XY. Histopathology of the excised gonads determined mixed germ cell tumor in right ovary and streak left gonad without gonadoblastoma in left side. In patients suffered from Swyer syndrome, high risk of gonadal neoplasia dictates early prophylactic gonadal excision to lengthen survival. Back to Top -
Palmar fasciitis and arthritis Syndrome associated with metastatic ovarian cancer: A paraneoplastic syndrome
Palmar fasciitis and polyarthritis syndrome (PFPA) is an uncommon syndrome that affects predominantly elderly women and characterized by symmetrical polyarthritis followed by flexion contracture of the hands. It is usually associated with a metastatic malignant neoplasm, and therefore implies a poor prognosis. We report a case of a 54 year old woman presented with palmar fasciitis and polyarthritis six months before the diagnosis of a metastatic adenocarcinoma of the ovary. Surgical excision of the tumor and adjuvant chemotherapy caused remission of the polyarthritis. Back to Top -
Trichilemmal Pilar Tumor of the Scalp: A Case Report
Proliferating trichilemmal tumors (PTTs) are uncommon exuberant growths of cells derived from the external root sheath. They tend to occur in older women, with a predilection for the scalp. Wide local excision has been the standard treatment. Recent reports have described a rare malignant variant with an aggressive clinical course and a propensity for nodal and distant metastases which, therefore, merits aggressive treatment. Back to Top -
Splenic parenchymal metastases from epithelial ovarian cancer: case report
Ovarian tumor spreads transcelomically, by lymphatic route and/or by hematogenous route. Splenic parenchymal metastases are very rare. Herewith we are presenting a case of primary ovarian tumor with metastases to the omentum, paraaortic lymphnodes and splenic parenchyma. These represent all the three routes of spread of primary ovarian tumor. The hematogenous spread as in this case to splenic parenchyma carries a poor prognosis as per small available literature. With optimal cytoreductive surgery, as in this case which has been carried out, a good prognosis may be expected. Back to Top -
Chemotherapy Treatment of metastatic breast cancer (MBC)
Metastatic breast cancer is not curable by current treatment modalities, although temporary regression of the disease is attainable in about 65% of the patients. Clinical complete remission is observed in less than 20% of the patients but rarely of long duration. Median survival is of about 2 years. The goals of the treatment therefore is to palliate the symptoms of the patients and if possible prolongation of useful high quality life. Surgery and radiation therapy play a limited role in patients with metastatic breast cancer as to make a histological diagnosis or mastectomy to prevent local complications. Endocrine therapy, chemotherapy, monoclonal antibody therapy and combination of these approaches have proved useful in the management of metastatic breast cancer. Since metastatic breast cancer is incurable today we must emphasize early detection and diagnosis of breast cancer and to continue clinical researches to improve the outcome of metastatic breast cancer. Back to Top -
Basal cell carcinoma of upper lid margin, case report
Basal cell carcinoma (BCC), is the most common form of skin epithelial cancer, adding up to 80-90% of skin malignancies, with a mortality rate less than 0.1%, with unknown survival rate. Sun exposure, (UV) is tumorigenic either by cellular transformation or cellular mutation. The cure rate is almost 95%, depending on; the histological staging of the disease, time of diagnosis, and treating modality. BCC is a locally disfiguring malignant tumor. Back to Top -
Castleman’s Disease - unicentric plasma cell variant - a case report with review of literature
Castleman’s disease (CD) is a rare disease with two identified forms. The localized form, often pauci symptomatic is characterized by enlargement of an isolated single lymphnode or lymphnode chain. Multicenteric form frequently presents with general signs. We report a patient who presented with cough and hemoptysis with imaging revealing right hilar lymphadenopathy. After thoracotomy, lymph node excision biopsy was done, histopathological examination revealed plasma cell - variant (PCV) of CD, a rare location for unicentric PCV of CD. Back to Top -
Primary Non-Hodgkin Lymphoma of Frontal Sinus diagnosed by Fine needle aspiration cytology
We present a rare case of primary non- Hodgkins Lymphoma of the frontal sinus diagnosed by Fine needle aspiration cytology (FNAC). FNAC is a safe, simple, rapid and effective technique that could be used to diagnose lesions even in unusual sites like paranasal sinuses with effective radiological guidance. Neoplasms of the frontal sinus could be easily misdiagnosed as an inflammatory process clinically. FNAC is a simple test to rule out a neoplasm. A review of a single case including radiographic, clinical, and pathologic findings was done, followed by a discussion on the pathological differential diagnosis highlighting relevant literature. Timely diagnosis and management is critical in the management of these cases. Back to Top -
Periosteal Osteosarcoma of fifth meatase: The first case report
Periosteal osteosarcoma (PO) is a rare type of chondroblastic osteosarcoma. This case is present for two reasons. The first purpose of this article is to describe an exceedingly rare type of osteosarcoma in an unreported location and the second is Medullary involvement is rare in periosteal osteosarcoma and this case is the 22th cases of PO with medullary involvement. Back to Top -
Osteosarcoma of the forearm - a rare case report
Osteosacoma of the forearm and hand is extremely rare and no thorough evaluation of the osteosarcoma involving the forearm only is available. An eleven year old boy having pain and progressive swelling of the lower one third of the right forearm. Open biopsy was performed and the histopathological examination of the pathologic specimen characterized the lesion as high grade osteoblastic osteosarcoma .The patient underwent neoadjuvent chemotherapy and through elbow disarticulation. Back to Top -
Carcinosarcoma of Renal Pelvis with Immunohistochemical Correlation
Carcinosarcoma, a malignant tumor with biphasic morphology is uncommon in the renal pelvis. Immunohistochemistry(IHC) plays an important role in establishing the diagnosis and differentiating it from other biphasic malignant tumors. We present a rare case of immunohistlogically confirmed carcinosarcoma of renal pelvis in a 42 year old female, which possibly developed on a background of multicentric squamous cell carcinoma arising as a consequence of chronic irritation caused by calculi. Back to Top -
Adenoma of the nipple in a man: Case report
Adenomatosis (adenoma, papillary adenoma, florid papillomatosis) of the nipple is a rare benign disorder involving the nipple witch can be mistaken clinically for Paget's disease and pathologically be misinterpreted as an adenocarcinoma. It occurs mostly in middle-aged women and is extremely rare in adolescents and children. Adenomatosis of the nipple is a well-known in women, but extremely rare in males. We report a 55-year-old man with an adenomatosis of the nipple evolving positively after surgery. Back to Top -
Obesity and Breast cancer in the State of Kuwait
Breast cancer is the most common cancer among females in the State of Kuwait. It is well known fact that obesity is one of the risk factor in the development of breast cancer especially in the postmenopausal women. Since large number of Kuwaiti female population is obese and overweight, a study was undertaken in Hussain Makki Juma Center for specialized surgeries between 1992 to 2002 consisting of 153 randomly selected patients among Kuwaiti females. There were 63 patients in the premenopausal group and 90 patients in the postmenopausal group. Body mass index was used to classify the nutritional status of these patients. The overall incidence of obesity in these patients was 64.70%. The majority of patients 64/90 were obese in the postmenopausal group and 35/63 obese in the premenopausal group. The conclusion of this study was that there was significant association of obesity and breast cancer among Kuwaiti women. Back to Top -
Shattering the underpinnings of neoplastic architecture in LNCaP: synergistic potential of nutraceuticals in dampening PDGFR/EGFR signaling and cellular proliferation
Objective: Prostate cancer is a polyfactorial molecular anomaly that is offering refractoriness against a broad range of therapeutic drugs. Growth factor receptors are actively implicated in oncogenesis. PDGFR/EGFR mediated exacerbated signaling has a central participation and is contributory in fueling the signal transductions that gear up prostate cancer progression. Materials and Methods: In this particular study, androgen sensitive, Prostate cancer cell line (LNCaP) was used. Pretreatment of cell line with PDGF resulted in an enhanced proliferation of cells which was evaluated by MTT assay. Treatment of cell line with either alone Curcumin, EGCG, sulforaphane or in combination was evaluated. PDGFR/EGFR activation (phosphorylation) was studied using western blot. Results: Results indicated that phosphorylation was gradually downregulated after treatment with individual compound. However there was a remarkable decrease in cellular proliferation after a combinatorial approach which is indicative of the fact that PDGFR phosphorylation was decreased outstandingly as evaluated by MTT assay. That also gave a prominent decline in the expression and subsequent decrease in proliferation pattern of cells. Conclusion: Despite the fact that little is still known regarding the mechanistic insights by which phytonutrients act as barrier to cancer, and attempts to translate the studies from benchtop to bedside are in progress. A detailed analysis of nutraceuticals will help a lot in identifying the stumbling blocks in the standardization of therapeutic interventions. Back to Top -
Towards TRAIL to checkmating FLIP by silencing of SMURF and NEDD4
Objective: Prostate cancer is a multifactorial anomaly that arises and exacerbates because of miscellaneous key mediators instrumental in disease aggressiveness. Heterogeneity of the disease offers stumbling blocks in standardization of therapeutic interventions. TRAIL and cFLIP are two diametrically opposed key mediators that antagonize each others function. TRAIL has central role in killing neoplastic cells and sparing innocent cells. However this ligand also undergoes a down regulation in prostate carcinogenesis. In this particular study we hypothesized whether abrogation of negative regulators of TGF signaling potentiates and abolishes the expression of TRAIL and cFLIP respectively or not. Materials and methods: In this particular study we have used androgen sensitive prostate cancer cell line (LNCaP) and treated it with TGF. TGF treatment was also given to LNCaP cell line. RNA interference technique was used to unfold the correlation between TGF signaling and TRAIL and cFLIP expression in the LNCaP cell line. The results were analyzed by RT PCR and western blot. Results: We have treated the TGF treated cell line with siRNA of NEDD4 and SMURF. There was a successful blockade of both genes at transcriptional level as evidenced by RT PCR study. Simultaneously there was remarkable upregulation in the expression of TRAIL. Another interesting observation was that TGF treatment triggered expression of TRAIL and ablated cFLIP and this activity was pronounced after abrogation of negative regulators of TGF signal transduction. Conclusion: In this study, we show that TRAIL expression is up regulated and cFLIP is down regulated upon exposure of LNCaP cell lines to TGF-β and etoposide and that TRAIL is a major contributor to apoptosis mediated by TGF-β. Consistent with the interpretations it is obvious that NEDD4 and SMURF are the major proteins involved in the deviation of core biological systems and combinatorial blockade of these genes by rational drug design or alternatively, drugging negative regulators of TGF pathway might offer exciting avenues in translational oncology. Back to Top -
Extensive review in the diagnosis of the malignant transformation of pleomorphic adenoma
Background and Objectives: There is an increasing likelihood of malignant change in pleomorphic adenoma with time. This extensive review will highlight some current areas of difficulty or controversy in diagnosis of the malignant transformation of pleomorphic adenoma. This is one of the most interesting and challenging issues for pathologist. Also we will compare the clinical and pathological point view for the diagnosis of malignant transformation of pleomorphic adenoma. Methods: A literature search using MEDLINE, accessed via the National Library of Medicine PubMed interface, searching for articles relating to malignant transformation of pleomorphic salivary adenoma written in English. Results: The update literature indicates that carcinomas in pleomorphic adenoma may arise in an older age group than benign lesions and are usually larger and longer standing lesions. with evidence of recurrence or a previous lesion. Although of the use the molecular changes in studying malignant transformation of pleomorphic adenoma but unfortunately, there is no specific expression for any of the used proteins in those tumour to determine the precise early diagnosis of malignant transformation of pleomorphic adenoma from pathological point view. Conclusions: The use of clinical evidence is more reliable than pathological evidence to diagnose malignant transformation of pleomorphic adenoma. Back to Top -
Mammary fibromatosis in a male breast
Mammary fibromatosis is a rare disease. It accounts for less than 0.2% of all primary breast lesions.Although several series of this condition have been reported in women, mammary fibromatosis in men is extremely rare. It is a relatively benign, though locally invasive neoplasm. It is usually misdiagnosed as breast carcinoma preoperatively. A review of the literature indicated that differentiation of fibromatosis from carcinoma is very difficult by radiological examination. We report the case of a 46-year-old gentleman with mammary fibromatosis. To the best of our knowledge, there are only few cases reported on male breast fibromatosis. The optimal management of it is unknown because of the rarity of the disease. Back to Top -
Strategy of surgical treatment of Giant presacral tumor: a case report
Presacral tumors are uncommon lesions that can be difficult to diagnose because of their nonspecific presenting signs and symptoms. Surgery is the mainstay of treatment as it establishes the diagnosis and prevents the adverse consequences associated with malignant degeneration and secondary bacterial infection. Large, highly vascular pelvic tumors are challenging to manage, bleeding as well as intraoperative tumor perforation are the major concerns of the surgeons. Cross-sectional imaging is essential in evaluating these lesions to determine the optimal surgical approach and the extent of resection. Here authors are emphasis a multidisciplinary and wise, expert individualized approach. We report a case of a presacral giant gastrointestinal tumor which was considered 30 months ago as unresectable, managed successfully by preoperative vascular embolization and abdomino perineral approach. Back to Top -
Comparison of Homogeneity Indices for Quantitative Evaluation of Dose Homogeneity for IMRT Treatments of Head and Neck Cancers
The purpose of this study was to evaluate different homogeneity indices for IMRT of head and neck cancers and to suggest the best representative homogeneity index for quantitative measure of dose homogeneity. In this study 3 different homogeneity indices were evaluated for 22 head and neck cancer patients receiving dynamic IMRT treatments on Clinac-DHX linear accelerator with 6 MV photon beam. IMRT planning was carried out with Helios software on Eclipse treatment planning system. H index, HI index and S index proposed by Yoon (2007) were calculated for these patients. The H-index, HI index and S index values varied between 1.024 to 1.112, 4.03 to 16.9 and 0.94 to 3.43 respectively. H index values for patient 5 and 10 are identical (H index-1.06) though different in DVH distributions but the S index values for these patients are different (1.36 and 2.01). Similarly the HI index for the patient 11 and 16 are identical in spite of different in DVH distributions but S index values are different for patient 11 and 16. Since the S index represents the whole DVH curve unlike the conventional indices which depends on dose at a point, it is the better method to quantify the dose homogeneity. These results indicate that H and HI indices do not provide the accurate dose homogeneity information, but the S indices uniquely provide quantitative information about the dose homogeneity. Back to Top -
Primary diffuse large B-cell lymphoma of the eyelid in a 70 year old female presenting as a huge swelling: A rare case with short review of literature
A 70 year old female presented with a swelling of the right upper eyelid with no associated history of trauma to the right eye. She was evaluated at our cancer Institute and excision biopsy was performed which was positive for malignancy. Immunohistochemistry confirmed the diagnosis of extra nodal malignant lymphoma, diffuse large B-Cell lymphoma. She received chemotherapy with R-CHOP protocol and has responded clinically and radiologically well to the chemotherapy after completion of six cycles of chemotherapy. Orbit and eyelids are extremely rare sites of primary large B-cell lymphoma and very few cases have been reported so far in the English medical literature. We report this rare case with a short review of literature. Back to Top -
Carcinosarcoma of renal pelvis, an oncological curiosity
Carcinosarcomas are rare biphasic malignant neoplasm with an epithelial and a spindle cell component. Primary renal pelvic carcinosarcoma is extremely rare. We present a case of 60-year-old male with primary renal pelvis carcinosarcoma. Back to Top -
Extra nodal non-hodgkin's lymphoma of the abdominal wall: A case report
Primary soft tissue lymphoma of the abdominal wall is an extremely rare entity. This report is about a patient who presented with a mass lesion of the abdominal wall which clinically and radiologically mimicked a soft tissue tumor. Histological examination of specimen obtained by complete surgical excision suggested diffuse large B-cell lymphoma. Subsequent clinical staging did not reveal involvement of any other sites by the disease. Thus our patient had Ann Arbor stage IA disease with an international prognostic score of 0.Post operatively the patient is being treated with immuno chemotherapy with cyclophosphamide, doxorubicin, vincristine and prednisolone regime. In this report the case has been discussed along with a brief review of relevant literature. Back to Top -
Progression or Deception? The Dilemma of Serum Tumour Marker (STM) Flares
The routine use of serum tumour markers (STM) to monitor response to chemotherapy is recommended in very few malignancies, but many more are used in clinical practice. In an ideal world, tumours and their markers are required to regress in a linear fashion signaling tumour response; any increase in values should be interpreted as a mark of failure and chemo-refractoriness. However, in the less than perfect world of oncology, tumours might actually enlarge in size on imaging, or tumour marker levels increase initially when exposed to chemotherapy drugs, only to subside later. The latter phenomenon is variously called “flare”, “surge”, “spiking” or ”pseudoprogression” and has misled many a medical oncologist into abandoning potentially effective treatment. This article is an attempt to remind the practicing medical oncologist about the phenomenon and to suggest an approach to the problem. Back to Top -
Inguinal Fungating Mass in a Case of Carcinoma Ovary : A Case Report
Cancer that forms in the tissue of the ovary is called ovarian cancer. Ovarian tumours, other than highly malignant sarcomas and teratomas which spread by the blood stream to the lungs, rarely produce remote metastasis. The lymph nodes most likely to be involved are the aortic group . The incidence of inguinal lymphadenopathy from ovarian carcinoma at presentation is rare. We present a case of 55 year old female patient who presented with right inguinal fungating mass in a case of carcinoma ovary with raised CA-125 levels and review of relevant literature. Back to Top -
Carcinoma Stomach: A Retrospective analysis from AIIMS, New Delhi
arcinoma of stomach was the leading cause of cancer related death in the last century worldwide. However in many parts of the world, the incidence has gradually decreased due to changes in food habit and the environment. The proximal gastric and gastro- esophageal junction (GE junction) cancer has markedly increased in the last three decades. The only proven curative treatment is surgery, but due to high recurrence rate, efforts are on regarding systemic and regional adjuvant therapies. The present study is retrospective analysis of all the post operative (post-op) cases of carcinoma stomach and the GE junction being registered and treated in our department from June 1995 to February 2008. The total number (no.) of patients (pts) were 69 (54 stomach, 15 GE junction), male 49 females 20, KPS 50-90 (median 80), length of lesion <5cm 28, transmural infiltration along with serosa involvement was found in 40 pts. Histologically, Adenocarcinoma was detected in 61 pts and metastatic spread in 50 pts. Thirty-six pts had surgery outside. Forty-nine pts had subtotal gastrectomy, while lymph nodes dissection was done in 64 pts. Chemo-radiotherapy (CTRT) was given in 49 pts, and 54 pts received >30 Gy radiation dose. Mc Donald’s regimen was used in 49 pts. Treatment compliance was found in 53 pts, only 05 pts hospitalized for supportive management. Pts were followed up from 01-69 months (median 12 months). Recurrence was found in 13 pts (median 14 months). The multimodality mode is better treatment option in the management of carcinoma of stomach. Back to Top -
Dosimetric Characteristics of Leksell Gamma Knife® 4C
To evaluate the measurement of dosimetric parameters for Leksell Gamma Knife® 4C (LGK 4C) with the calculations performed using Leksell Gamma Plan. All measurements were carried out by using ELEKTA supplied spherical ABS (acrylonitrile butadiene styrene) plastic phantom with the diameter of 160 mm. The irradiation geometry consisted of the placement of a single shot at the center of this phantom (i.e.) X = Y = Z = 100 for all collimators of 4, 8, 14 and 18 mm. Gafchromic EBT type films were used to perform the measurements and Leksell Gamma Plan (LGP) version 5.34 was used to perform the calculations. The obtained parameters were then compared. The investigated dosimetric parameters were dose profiles for all collimators in all 3 axes (X, Y, and Z) including the full width at half maximum (FWHM) and the penumbra for each profile and also the relative output factors for all collimators. The exposed films were scanned by means of a flatbed scanner (EPSON Expression 1680 Pro) with the selected geometrical resolution of 0.05 mm. Measured Dose Profiles on 3 axes for all collimators agreed very well with the Leksell Gamma Plan calculated profiles within the specifications (≤ 1 mm at 50 % isodose level). There was excellent agreement between the experimental and calculated parameters of FWHM & penumbra for all collimators. Also, the measured relative output ratio of 14, 8 and 4 mm collimators was 0.986, 0.975, and 0.849 against the default values (0.984, 0.956, and 0.87) entered by vendor in the planning system. Our experimental results are very well matches with LGP calculated parameters. Back to Top -
A simple method for evaluating stereotactic accuracy of Magnetic Resonance Imaging and Computed Tomography Imaging in Frame Based Radiosurgery
Purpose: To design a simple direct and reproducible method with minimum uncertainty for evaluating the stereotactic accuracy of Computed Tomography imaging and Magnetic Reso-nance Imaging and to compare the results. Methods: 3Dphantom has been designed with 1 mm diameter targets in eight sectors for stereo-tactic MR imaging and CT imaging. The phantom was connected to Leksell stereotactic frame and the images obtained are exported to treatment planning system.Leksell stereotactic coordi-nates (X, Y, Z) of the holes are determined in each slice and mean maximum errors were calcu-lated along with 3D vector distances from centre of stereotactic coordinate system (100,100,100) to five known Targets. The target volumes are calculated independently for each image data sets. Results: The mean of maximum absolute error estimated for MR images from the Siemens Magnetom vision MR unit were 0.46 mm (X-Axis), 1.66 mm (Y-Axis) and 2.11 mm (Z-Axis). Mean of absolute maximum error estimated using CT images from the Philips Brilliance 16 CT scanner were 0.29mm (X-axis), 0.51mm (Y-Axis) and 0.90mm (Z-Axis). 3D vector calculations were 0.38 mm for CT and 0.72 mm for MRI. No significant variation is noticed in volume calculations from both image data set. Conclusion: This study showed accuracy and quality of stereotactic CT- imaging and MR-imaging are limited by localization devices and their designs in frame based Radiosurgery. This method is simple direct and economical with good resolution and high reproducibility. Back to Top -
Non Hodgkin's lymphoma presenting as ileal Intussusception
Intussusception is a pathology in which telescoping of a proximal segment of bowel occurs into lumen of the distal segment. Intussusception is a rare cause of intestinal obstruction in adults. And Non Hodgkin's lymphoma (NHL) is not commonly found to be a lead point in intussusception. Hence, with high suspicion of lesions, considering the age and the bowel involvement, resection of the diseased bowel may be the single most important decision in salvaging these patients. Here we report a 55 yr old female patient presenting with acute intestinal obstruction due to Non Hodgkin's lymphoma (NHL) causing intussusception. Back to Top -
Different Surgical Modalities in Management of Paediatric Abdominal Lymphoma
Background: The abdomen is one of the most frequent sites for lymphoma in children. The role of surgery has been limited to intra-abdominal respectable tumours or as a diagnostic procedure in case of disseminated disease. Laparotomy without total excision of the tumour does not improve survival; moreover, it may cause complications and delays initiation of chemotherapy. Aim of the work: This study was undertaken to assess the role of surgery in the management of children and adolescents presenting with intra-abdominal lymphoma in order to create certain criteria to select the proper surgical modality for managing those patients. Patients and Methods: This retrospective, descriptive study was done on 33 patients of abdominal lymphoma over a period of seven years from 2000 to 2007. Patients’ files were reviewed regarding the full clinical examinations, laboratory and radiological investigations as well as surgical and diagnostic procedures. Collected data were tabulated and statistically analyzed using SPSS program package. Results: Patients’ ages ranged from 2.5 to 16 years with a mean of 6.7. They were 18 females and 15 males at F/M ratio 1.2: 1. In those patients who underwent surgery; seventeen (51.5%) presented with emergency complaints, 10 patients (30%) with acute abdominal pain and 7 patients (21.5%) with intestinal obstruction with surgical excision of the tumour mass. In the remaining 16 patients, 9 had huge pelvi- abdominal masses and 7 had generalized lymphadenopathy where the intra-abdominal involvement was discovered following further investigation. Conclusion: Surgery still has a role in treatment of lymphoma whether non Hodgkin or Hodgkin's as complete resection does improve the survival rate, however, in disseminated of metastatic disease, aggressive debulking of the tumour should be avoided as chemotherapy is to be instituted primarily. Surgical resection does not cause significant change in morbidity or mortality. Back to Top -
Primary adenoid cystic carcinoma of the breast: Case report and review of the literature
Adenoid cystic carcinoma of the breast is a very rare neoplasm. We report a case of adenoid cystic carcinoma of the right breast presented with painless lump in the upper outer quadrant managed with lumpectomy, axillary lymph node staging and adjuvant local external radiotherapy to the whole breast with simultaneous integrated boost to the site of primary disease using respiratory gated intensity modulated radiotherapy. The available literature is reviewed. Back to Top -
Coagulation abnormalities in Type 1 Gaucher disease is a cause of bleeding tendency and can be restored by Enzyme Replacement Therapy
In type 1 Guacher disease a bleeding tendency occurs which is partly caused by thrombocytopenia due to massive splenomegaly. In addition, low levels of factors IX and XI have been described. The mechanism responsible for these clotting factor abnormalities is unknown. We performed a detailed study of parameters of coagulation, anticoagulant and fibrinolysis on 2 patients diagnosed as type 1 Guacher disease. Patient 1 is treated by enzyme replacement therapy (ERT) while patient 2 (historic control) does not receives treatment. For patient 1 the coagulation, anticoagulant and Fibrinolytic parameters were measured before, after 6 months and after 12 months of ERT, while for patient 2 they were measured once at the time of study. We found that before or without treatment most of the coagulation parameters (PT, PTT, Factors V, VII, IX, X) were abnormal but by different severity and the most affected factors were (factors VII, X), in addition, to deficient anticoagulant parameters (protein C and protein S). Markers for activation of fibrinolysis (D-dimer, FDP) were significantly elevated. Most of these coagulation, anticoagulant and Fibrinolytic parameters were corrected after treatment by ERT. Thus, severe disorders of the coagulation system occur in Gaucher disease, contributing to the bleeding tendency. Our results suggest that low grade ongoing activation of coagulation and balanced by simultaneous activation of the Fibrinolytic system may be responsible for subsequent depletion of coagulation factors, anticoagulant and Fibrinolytic parameters possible due to mononuclear cell activation. Enzyme replacement therapy restores most of these parameters to normal level within 6 – 12 months. Keywords: Gaucher disease, coagulation disorder, clotting factor deficiencies, enzyme replacement therapy. Back to Top -
Dosimetric consideration of transient volume enlargement induced by edema in prostate brachytherapy seed implants
Purpose: To investigate enlargement of prostate volume by edema during brachytherapy seed implantation and develop a nomogram model to calculate air-kerma strength (AKS) required for implantation of the enlarged transient prostatic volume. Materials and Methods: The prostate volume was measured prior and after seed
implantation using trans-rectal ultrasound imaging in the operating room to obtain volume enlargement. A nomogram model was developed that calculates AKS required for implantation of the enlarged transient prostate volume with optimal dose coverage. Results: The measured prostate enlargement in this study was up to 60% of the initial volume. The effective prostatic volume enlargement was calculated for three isotopes: 125I, 103Pd and 131Cs. The effective volume enlargement for 125I implants was relatively small (< 10%) because of its long half-life. For 103Pd and 131Cs with short half-lives, additional AKS up to 20% and 30%, respectively, might be required to provide appropriate dose coverage of possible enlarged prostatic volumes. Conclusions: Prostate volume enlargement should be considered to obtain optimal dose coverage particularly for short half-life isotopes such as 131Cs and 103Pd. The nomogram model developed in this work provides the AKS required for implants with a wide range of prostatic volume enlargements (5-100%) for three isotopes. Key words: prostate brachytherapy; nomogram; air-kerma strength; edema; volume enlargement. Back to Top -
Primary squamous cell carcinoma - very rare malignancy of the breast
In females, the most common cancer is breast carcinoma in which squamous cell carcinoma is a rare type of malignancy. Histologically, infiltrating ductal carcinoma is the most common type and lobular, mucinous and medullary types have lower incidence. Pure squamous cell carcinoma of the breast can originate from the skin, nipple or epithelium of a deep-seated dermoid cyst or squamous metaplasia on chronic inflammation background. We are reporting a rare case of primary squamous cell carcinoma of the breast. In follow-up of 8 months, patient is doing well. We discussed our approach for treatment with review of the literature. We have treated this patient successfully with surgical and adjuvant chemotherapy. Keywords – breast; surgery; squamous carcinoma; chemotherapy. Back to Top -
Prominent Intracytoplasmic Crystals In Alveolar Soft Part Sarcoma: An Aid In Cytological Diagnosis
Alveolar soft part sarcoma is a rare neoplasm of unknown histogenesis with poor prognosis. Due to the epithelioid appearance of the neoplastic cells, ASPS may resemble many neoplastic conditions, such as metastatic renal cell carcinoma, granular cell tumor, epithelioid sarcoma and melanoma. Presence of abundant, rod like crystals in the cytoplasm of tumour cells is an important finding characteristic of this tumor, which helps in differentiating it from the others. In this case study, we have highlighted cytological features that help in diagnosis, like the background, cell morphology and the crystals. Key words: Intracytoplasmic crystals, dirty background, binucleate cells.
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Low Grade Follicular NonHodgkins lymphoma of bone and Ovary- Unusual presentation and review of literature
Primary Nonhodgkins lymphoma of the bone or ovary is rare. They are usually involved secondarily to generalised lymphoma. The diagnosis is usually delayed because of nonspecific clinical signs and equivocal radiologic findings. The pathology of bone/ ovarian NHL tends to be of the aggressive type especially when secondary involvement is seen. We report a rare case of low grade follicular lymphoma involving only the ovaries and multiple bones. The patient did not have any other organ involvement or lymphadenopathy by clinical examination or CT scans. The patient was treated with chemotherapy in view of disseminated involvement.
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Limb sparing surgery in soft tissue sarcoma of extremities: An Indian Perspective
This is a retrospective study of 57 patients with extremity soft tissue sarcomas treated at Gujarat Cancer Research Hospital, Gujarat (India) from January 2005 to December 2007. The aim of this study is to review the feasibility of limb salvage surgery in extremity soft tissue sarcoma in Indian population. The mean follow-up time was 25 months. Twenty eight percent of tumors were located in upper and 72% in lower extremity. The goal of treatment was limb preservation if feasible. The treatment consisted of amputation in 17 (30%) and local wide excision in 40 (70%) patients. The commonest histology was pleomorphic sarcoma (21%). Sixty four percent patients were treated with surgery alone, 32% with surgery plus radiation & 4% with surgery plus systemic chemotherapy. Inadequate margins were present in 12% of the patients. All the patients were followed up for atleast 2 years. Recurrence in the form of local and distant occurred in 39% of the patients. Local recurrence occurred in 18% of the patients who underwent local wide excision and in 35% of those who underwent amputation. Pulmonary metastases were detected in 16% of patients. The 2 year mortality was 10.5% with the most common cause being lung metastasis and the most common histology being leiomyosarcoma. We concluded that limb salvage surgery for soft tissue sarcoma is feasible in our population (70%) although the rate is lower than that of world literature (90%).
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Dosimetric Comparison of Carcinoma of Breast Treatment Plans using Respiratory Gated IMRT In Inspiratory Breath Hold Versus 3DCRT
Background and purpose: There are various techniques of radiation therapy for breast cancer treatment. Each technique has its advantages and disadvantages. Chest wall radiotherapy is conventionally employed by two tangential along with one direct field in most of the centers, even if issue of Inhomogeneity, junction dose and chest wall moment during respiration remains concern. In recent years 3DCRT is employed widely over conventional technique that improved the junction doses but doses to Organ at Risk still be minimized. A few clinical studies have come using IMRT to address these shortfalls. This study aimed to compare the treatment plans of Respiratory Gated IMRT versus 3DCRT for chest wall radiotherapy in breast cancer patients.
Materials and Methods: 3DCRT treatment plans were created for consecutive 27 patients who were treated earlier with Respiratory Gated IMRT. Multiple beam 3DCRT plans were created for same volumes used for IMRT. Dose was prescribed as 45 Gy in 25 fractions to the PTV. Treatment plans were normalized to 45 Gy to 95% of the PTV. The Conformity, Homogeneity, Lung dose, Heart dose, LAD dose, Low dose volume and Integral dose were recorded and analysed.
Results and Discussions: The Target coverage is achieved 95 % of prescription to the 95% of the PTV in both the plans. Lung V20 mean is 53.2 % for 3DCRT and 29.5 %( p <0.0001) for IMRT. Heart V40 mean is 4.57% for 3DCRT and 0.49% (p=0.0006) for IMRT. For Left sided breast cancer patients Heart V40 for 3DCRT is higher than IMRT (p=0.0273). Both Lung V20 mean is 27.2% for 3DCRT and 15.19 (p<0.0001) for IMRT.LAD maximum Dose to left side breast patients (Mean) is 37.32 Gy for 3DCRT and 27.81 Gy (p=.0048) for IMRT. Monitor Unit Mean is 208 for 3DCRT and 1261(p<0.0001) for IMRT.
Conclusion: IMRT reduces the maximum doses (Dmax) to target volume. The normal tissue dose also reduced significantly with IMRT. Even though 3DCRT provided good target coverage, normal tissue sparing was lesser degree with compared to inverse planned IMRT plan. The probability of occurring pneumonitis, cardiac toxicity and skin reaction is expected to be less with IMRT technique. However the high integral dose and high monitor unit remains an issue of concern.
Keywords: Intensity Modulated Radiotherapy (IMRT), Three Dimensional Conformal Radiotherapy (3DCRT), Respiratory Gated IMRT -
Hemangiopericytoma of the forearm: A case report and review of the literature
Hemangiopericytomas are rare soft tissue tumors of the vascular origin. They occur in any organ but most frequently have been reported in the extremities. We report a case of hemangiopericytoma of the left forearm treated with optimal debulking surgery followed by postoperative adjuvant external beam radiotherapy using intensity modulated radiotherapy (IMRT). The value of recurrence free survival after adjuvant radiotherapy is discussed .Currently patient is free of disease and disability at a mean follow up period of 4 years. Key Words: Hemangiopericytoma, surgery, adjuvant radiotherapy -
Solving the Puzzle
Castleman’s disease (CD) is a rare entity that requires a high level of suspicion. The diagnosis of our patient was a challenge after extensive work up the diagnosis was delayed. The diagnosis of CD was missed in the first reading of mesenteric lymph nodes. It is worth to review patient’s laboratory, radiological and histopathology results retrospectively to be sure that the diagnosis is not missed. Keywords: Castleman’s disease, lymphoproliferative disorders -
Penile cancer in India: A clinicoepidemiological study
Introduction: Penile cancer is one of the most common genitourinary cancers encountered in developing countries like India. The incidence of carcinoma of the penis varies according to circumcision practice, hygienic standard, phimosis, number of sexual partners, HPV infection, exposure to tobacco products, and other factors. The aim of the study was to study the clinic-epidemiological profile of penile cancer. Materials and methods: 54 patients were enrolled and studied from May 2005 to June 2006. Clinicoepidemiologic profile and patients characteristics were assessed in a questionnaire including demographic data, presence of risk factors like smoking, phimosis, circumcision, history of promiscuity, history of sexually transmitted disease etc., clinical features, pathological features of tumor like size, site, appearance, histological type, grade, presence of lymph nodes , treatment performed and response. 5 year survival rates were also calculated. Results: Of the 54 patients, about three-fourths patients were older than 50. About one quarter patients presented with phimosis and 20% patients had undergone circumcision after attaining adulthood. 70% patients were found to be smokers. Patients mostly presented with T2 disease whereas lymphadenopathy, both benign and malignant, was present in 55.5% and 76% patients in T1 and T2 patients respectively. 5 year survival rates were found to be 87% and 60% respectively in stage I and II respectively. Conclusion: penile cancer is one of the most common genitourinary cancer affecting mostly aged, uncircumcised males with history of smoking. Patients mostly present in advanced age and hence it calls for a national awareness campaign against this disease. Keywords: penile cancer, circumcision, phimosis, HPV, sexually transmitted diseases -
An unusual variant of prostatic adenocarcinoma with metastases to testis.A case report
Ductal adenocarcinoma of the prostate is considered to be a rare variant of prostatic adenocarcinoma when compared to the more common acinar adenocarcinoma. We report here a case of ductal adenocarcinoma of the prostate in a 68 year old man who presented with complaints of abdominal pain,retention of urine and hematuria of one month duration. Clinical examination showed prostatomegaly.The serum Prostate Specific Antigen [PSA] value was raised to 79ng/ml. Histopathological and immunohistochemical evaluation of resected specimen of prostate revealed ductal adenocarcinoma of the prostate.The patient was lost to follow up and presented four years after the initial diagnosis with metastasis to bone and testis.Though prostatic cancers have the ability for wide spread dissemination,metastasis to testis is rare.Immunohistochemical staining with PSA and Prostatic Acid Phosphatase [PAP] can help in establishing prostatic nature of the neoplasm. We are reporting this case because of the rarity of metastasis of prostatic carcinoma to testis and for stressing the need for keeping in mind the possibility of metastatic carcinoma also while dealing with testicular tumors. KEY WORDS: Prostate, ductal adenocarcinoma,metastasis, testis -
A case of isolated cutaneous Rosai-Dorfman-Syndrome
Rosai-Dorfman Syndrome (RDD) is a rare, benign, self-limiting disorder with is characterized by the non-malignant proliferation of distinctive histiocytic cell within lymphatic system. RDD has been described as a dynamic entity in the spectrum of histiocytosis with non-Langerhans cell histiocytosis at one end and Langerhans cell histiocytosis at the other. The exact etiology of this disease is uncertain despite widespread search for infectious or immunological reasons. We present a case of purely cutaneous Rosai-Dorfman Syndrome presenting as abdominal wall swelling. Keywords: Rosai-Dorfman Disease, Lymphoma -
Carcinoma of the breast in a young female – a rare case report
A 25 year – young female reported with a swelling in left breast since 4 months. She was lactating. Swelling was of small in size and increased to the present size, without any complaint of pain, loss of weight, appetite. There was no family history for breast disease. On examination, lump was present in the left breast in upper, outer quadrant region. Nipple was inverted and breast was enlarged in shape as compare to right breast with normal appearance of the skin. Lump was of size 10x8 cm, hard in consistency and mobile(figure-1,2). Non tender, axillary lymph nodes were felt only on left side. On pressing breast, lactations were present(figure-3). On fine needle aspiration cytology, diagnosis made as breast carcinoma. Modified radical mastectomy with axillary clearance was done with primary closure of the skin(figure-4, 5,6). Gross cut section of the specimen revealed large tumour of about size 10 x 12 cm with whitish in colour (figure-6). -
Analysis of low dose level volumes in intensity modulated radiotherapy and 3D conformal radiotherapy
To analyze the low dose volume regions in four different facets (1) Low dose volume regions are compared between 3DCRT and IMRT plans for each case, (2) Analysis of the effect on low dose volumes in 3DCRT as the number of fields is increased, (3) same study is done for IMRT and (4) The above two analysis are compared between the two modality. About 18 patients of different clinical diagnosis were taken for which both 3DCRT and IMRT plans with varying number of beams were performed. The plans were analyzed on the basis of conformity index and dose to the critical structures. In the first study, 5Gy volume region was greater for IMRT than for 3DCRT but the 10Gy, 15Gy and 20Gy volume regions were smaller for IMRT plans. In the second analysis as the number of fields increases the low dose volume regions also increases. In the third analysis also the same effect similar to second analysis is observed and finally in the fourth analysis the effect pronounced due to increase in the beam portals is similar in 3DCRT as well as IMRT. However from the fourth analysis it is also seen that with same number of beams, the low dose volume region is higher in 3DCRT than IMRT. A detailed analysis of the low dose volume regions is important in 3DCRT and IMRT plans with multiple beams which might lead to secondary cancer incidence. Here it is concluded that by increasing number of beams the low dose volume regions increases in both the techniques 3DCRT and IMRT. The study also shows that the 5Gy volume regions are slightly higher in IMRT compared to 3DCRT however with proper optimization the volume of tissue receiving 10Gy, 15Gy and 20Gy can be reduced. Key Words: Intensity Modulated Radiotherapy (IMRT), Three Dimensional Conformal Radiotherapy(3DCRT), Treatment Planning System(TPS), Low Dose Regions. -
Main determinants of severe neutropenia in patients with solid tumors receiving adjuvant chemotherapy
Chemotherapy-induced neutropenia as a major toxicity of systemic chemotherapy is commonly associated with substantial mortality and morbidity and thus identifying its-related determinants is necessary. This study identified main consequences of severe neutropenia following adjuvant chemotherapy treatment in a community-based population of patients with cancer in Iran. This prospective study included 828 consecutive patients with histologically proven primary or metastatic solid tumors and received chemotherapy. Demographics data, disease characteristics, and co morbidities were collected by interviewing with the patients and their laboratory data and files. Patients underwent complete blood count a week after the first chemotherapy course. Base on the absolute neutrophil count (ANC) nadir value, 30 patients (3.6%) had severe neutropenia. Multivariable logistic regression analysis showed that advanced age (OR = 5.262, p = 0.012) and the presence of diabetes mellitus (OR = 8.126, p = 0.015) were main determinants of the appearance of severe neutropenia with the presence of demographics and studied co-morbidities as confounders. We identified advanced age and the presence of diabetes as main determinants of high-grade neutropenia in solid tumor patients receiving adjuvant chemotherapy in the Iranian population. Keywords: Neutropenia, tumor, chemotherapy, metastasis, blood -
Intensity Modulated Radiotherapy (IMRT) in head and neck cancers – an overview
Radiotherapy (RT) is effective in head and neck cancers. Following RT, dryness and dysphagia are the 2 major sequelae which alter the quality of life (QOL) significantly in these patients. There is randomized evidence that Intensity Modulated Radiotherapy (IMRT) effectively spares the parotid glands. IMRT has been attempted in all head and neck sub-sites with encouraging results (discussed below). Role of IMRT in swallowing structure (constrictor muscles) sparing is less clear. Further improvement in results may be possible by using functional imaging at the time of RT planning and by image guidance/verification at the time of treatment delivery. The following text discusses these issues in detail. Key words: Head and neck cancer, IMRT
