A Rare Malignant Transformation of an Ovarian Cystic Teratoma: A Case Report.

Mature cystic teratoma (MCT) is the commonest germ cell neoplasm of the ovary but malignant transformation is a rare occurrence (1-2%). Of these malignancies documented in literature the commonest are squamous cell carcinoma and adenocarcinoma. Urothelial carcinomas arising in an MCT are a rare occurrence and only 7 cases have been reported in literature. We report a case of an MCT which was complicated by the presence of urothelial carcinoma confirmed on histopathological examination.

Investigation into celiac disease in Indian patients with portal hypertension.

BACKGROUND: There is limited data on celiac disease in patients with cryptogenic cirrhosis or idiopathic noncirrhotic intrahepatic portal hypertension (NCIPH). Our objective was to evaluate for celiac disease in patients with portal hypertension in India. METHODS: Consecutive patients with portal hypertension having cryptogenic chronic liver disease (cases) and hepatitis B- or C-related cirrhosis (controls) were prospectively enrolled. We studied tissue transglutaminase (tTG) antibody and duodenal histology in study patients. RESULT: Sixty-one cases (including 14 NCIPH patients) and 59 controls were enrolled. Celiac disease was noted in six cases (including two NCIPH patients) as compared to none in controls. In a significant proportion of the remaining study subjects, duodenal biopsy showed villous atrophy, crypt hyperplasia, and lamina propria inflammation, not accompanied by raised intraepithelial lymphocytes (IELs); this was seen more commonly in cases as compared to controls. An unexpectedly high rate of tTG antibody positivity was seen in study subjects (66 %) of cases as compared to 29 % in controls (p-value < 0.001), which could indicate false-positive test result. CONCLUSION: In this study, 10 % of patients with unexplained portal hypertension (cryptogenic chronic liver disease) had associated celiac disease. In addition, an unexplained enteropathy was seen in a significant proportion of study patients, more so in patients with cryptogenic chronic liver disease. This finding warrants further investigation.

Primary extrauterine endometrial stromal sarcoma: Located in pelvic and abdominal tissue and arising in endometriosis.

Primary extrauterine endometrial stromal sarcoma is a rare tumor and it is infrequently associated with endometriosis. We are reporting a case of this unusual tumor in a 42-year-old female who presented with multiple nodules of tumor in the abdomen and pelvis and with metastases in para-aortic lymph nodes. The right parametrium, in addition, had a focus of endometriosis, which was contiguous with the tumor, confirming its origin.

Eosinophilic disorders of the gastrointestinal tract: imaging features.

Eosinophilic disorders of the gastrointestinal tract are increasingly rare but remain an important cause of long-standing gastrointestinal symptoms. Diagnosis is usually delayed because the disease mimics other inflammatory disorders and is often not suspected initially. We report a series of four cases to highlight the various imaging appearances of this condition. Two patients presented with upper gastrointestinal involvement, one patient presented with small and large bowel involvement, and one patient presented with diffuse involvement of the entire gastrointestinal tract.

Eosinophilic esophagitis and pharyngitis presenting as mass lesion in a patient with inactive rheumatoid arthritis.

We describe here a case of longstanding rheumatoid arthritis (RA) presenting with recurrent episodes of epigastric pain, melena, nonprogressive dysphagia, and hoarseness associated with persistent peripheral blood eosinophilia. Her RA was clinically inactive, but she had significant lymphadenopathy and hepatosplenomegaly. Computed tomographic scan of the thorax revealed circumferential wall thickening extending from the oropharynx to the gastroesophageal junction with a large polypoidal mass projecting into the lumen of the stomach. Histology revealed infiltration of the esophageal mucosa by eosinophils with a density of 40 to 80 per high-power field. The stratified squamous epithelium of the pharyngeal mucosa was also infiltrated by eosinophils with a density of more than 100 per high-power field. Eosinophilic esophagitis and pharyngitis were diagnosed, and the patient was administered corticosteroids and hydroxyurea, following which her symptoms resolved. On repeat imaging, there was significant reduction in esophageal wall thickening and luminal dilatation. There are few reports of tissue eosinophilia in association with RA, but the pathogenesis and any definite association with RA are not clear.

Gastrointestinal lymphomas: pattern of distribution and histological subtypes: 10 years experience in a tertiary centre in South India.

BACKGROUND AND AIM: Gastrointestinal tract (GIT) is one of the major sites of extra-nodal lymphomas constituting 10-15% of all non-Hodgkin's lymphoma cases and about 30-40% of extra-nodal lymphomas. Considerable variation exists in the literature with respect to incidence of the various histological subtypes and sites of involvement. This study was undertaken to ascertain the anatomic distribution, histological subtypes and sites of all GIT lymphomas presenting to a tertiary referral hospital in southern India. MATERIALS AND METHODS: The histological material of 361 patients over a period of 10 years (2001-2010), with histopathological diagnosis of lymphoma involving the GIT (both primary and secondary), was analyzed retrospectively. All lymphomas were reclassified according to the World Health Organization 2008 classification. RESULTS: These 361 cases include 336 primary and 25 cases of lymphomas, where the involvement was secondary. Primary lymphomas consisted of 267 males (79.64%) and 68 females (20.24%) with a male:female ratio of 3.93:1. The mean age was 45 years (range 3-88). Diffuse large B-cell lymphoma (DLBCL) was the commonest subtype (222 cases; 66.71%), followed by low-grade marginal zone lymphoma of the mucosa associated lymphoid tissue (MALT) type (34 cases; 10.12%) and Burkitt's lymphoma (35 cases; 10.48%). The commonest site was stomach (180 cases; 53.57%), followed by small intestine (79 cases; 23.51%) and large intestine (68 cases; 20.23%), respectively. There were some uncommon types of GIT lymphomas documented during the study. CONCLUSION: In this largest retrospective single centre study from India, we establish that the pattern of distribution of primary GIT lymphomas (PGLs) in India is similar to the western literature in that the stomach is the commonest site of PGL and DLBCL is the commonest histological subtype. Immunoproliferative small intestinal disease cases were seen in this study, which is uncommon in the west.

Gastrointestinal stromal tumors: a 7-year experience from a tertiary care hospital.

BACKGROUND: Gastrointestinal stromal tumor (GIST), now the most common mesenchymal tumor of the gastrointestinal tract (GIT), has been frequently studied, especially with regard to its successful targeted therapy using imatinib mesylate. AIM: Our aim was to describe the clinicopathological features of a large number of cases from a tertiary care hospital in India and report on the follow-up after treatment of some of the cases, comparing them with series described in the west. DESIGN: This is a retrospective study of cases encountered over a 7-year period (1999-2005). RESULTS: Ninety-two cases of GIST were studied, which made up the largest group (52.8%) of mesenchymal tumors of the GIT, with smooth muscle tumors comprising 38.1%, the next large group. GISTs were almost equally prevalent in the stomach and the small intestine, unlike in most studies where stomach is the most common site. GIST may be considered as a cause of bleeding when upper and lower GI endoscopy is normal. Ninety-five percent of the GISTs were positive for CD117 (KIT), as is known. A majority of them (70.4%) were of the high-risk malignant category, unlike most studies where high-risk tumors make up 30-45%. Histologically, the majority had a pure spindle cell morphology and skenoid fibers were rare. Follow-up of 11 cases, the majority with high-risk tumor, treated with adjuvant imatinib for 6 months after surgical resection showed stable disease for periods from 2 to 5 years. However, 11 cases treated with imatinib for longer than 6 months had a poorer outcome due to recurrent, metastatic, or inoperable disease. CONCLUSION: In our study of a large number of GISTs, which were equally prevalent in the stomach and small intestine, the majority were of the high-risk malignant category and of pure spindle cell morphology. Limited numbers had follow-up after imatinib therapy, which showed in one group treated for 6 months, after resection of high-risk GIST, stable disease for periods ranging from 2 to 5 years. Molecular studies and larger numbers are required for meaningful conclusions to be drawn.

Primitive neuroectodermal tumor of the jejunum; a case report and literature review.

OBJECTIVE AND IMPORTANCE: The aim of this paper is to report an unusual presentation of extranodal follicular dendritic cell tumor of neck with spinal metastasis. Follicular dendritic cells are nonlymphoid immune accessory cells present in the germinal centers of lymphoid follicles and play a crucial role in the induction and maintenance of the humoral immune response. Tumors from these cells are rare and treatment modality poorly defined. CLINICAL PRESENTATION: A 37-year-old lady presented with recurrent neck swelling which was initially reported as malignant paraganglioma. The primary disease was treated with surgery and radiotherapy. Eleven years later, the patient presented with metastasis to the spinal cord. Subsequent immunohistochemical analysis of the primary site tumor and the metastatic deposits revealed it to be a follicular dendritic cell tumor. INTERVENTION: The patient was treated with surgery followed by radiotherapy to spine, and one and half year after treatment, the patient is doing well and has regained complete motor functions. CONCLUSION: Metastasis to spinal cord for follicular dendritic cell tumor is very rare, and to the best of our knowledge, no such case has been previously reported in the scientific literature so far. In the present case, good local control was achieved with initial surgery and radiotherapy but resulted in distant failure after 11 years. This underlines the need for adjuvant systemic therapy, and understanding the biology of the tumor may help in formulating targeted therapy in the future for this rare disorder.

Cervical embryonal rhabdomyosarcoma and ovarian Sertoli-Leydig cell tumour: a more than coincidental association of two rare neoplasms?

A case in which an embryonal rhabdomyosarcoma of the cervix and an ovarian Sertoli-Leydig cell tumour of intermediate differentiation occurred in a 13-year-old girl is described. Although initially considered as a chance association, a review of the literature showed the co-occurrence of these two uncommon neoplasms in three previous cases. The reason for this association, which is thought to be more than coincidental, is not known, although an underlying genetic abnormality is a possibility. The ovarian tumour in this case was characterised by the presence of foci of cells with extremely pleomorphic nuclei, which initially raised the possibility of metastatic rhabdomyosarcoma. These were interpreted as foci of bizarre nuclei within the Sertoli-Leydig cell tumour.

Fine-needle aspiration cytology versus open biopsy for evaluation of chronic epididymal lesions: a prospective study.

OBJECTIVE: To compare the efficacy of fine-needle aspiration cytology (FNAC) with that of open biopsy in the evaluation of chronic epididymal lesions. MATERIAL AND METHODS: Forty men evaluated for chronic epididymal lesions were studied. Men aged >25 years who did not intend to father any more children and had had local symptoms for >6 weeks were included. Of these 40 men, 23 who satisfied the above criteria were subjected to FNAC and open biopsy. The slides were reviewed by two independent pathologists. The sensitivity, specificity and positive predictive value of FNAC were compared with the results of open biopsy. RESULTS: Tuberculous epididymitis was the commonest cause of chronic epididymal lesions, followed by non-specific epididymitis, sperm granuloma, epdidymal cyst and normal epididymis. The sensitivity and specificity of FNAC for the diagnosis of tuberculous epididymitis were 87% and 93%, respectively. For the diagnosis of non-specific epididymitis, FNAC was 100% sensitive and 100% specific. The positive predictive value was 87% for the diagnosis of both tuberculous and non-specific epididymitis. CONCLUSIONS: FNAC has an overall sensitivity and specificity of 90% for diagnosing chronic epididymal lesions, with a positive predictive value of 87.5%. It is a valuable tool for evaluating chronic epididymal lesions. We suggest that FNAC should be the first-choice investigation for establishing the histopathological diagnosis of epididymal lesions.

Risk of colorectal cancer in ulcerative colitis in India.

BACKGROUND: The risk for colorectal cancer (CRC) in ulcerative colitis (UC) in India is not known. METHOD: Retrospective cohort from a tertiary level hospital in South India. Analysis of archived records of all patients with UC who underwent colonoscopy and segmental biopsies over the last 25 years. Incidence densities and risk of developing high grade dysplasia or CRC was calculated and chi-squared test was performed for risk factors of interest. RESULTS: Complete records were available for 532 patients, 336 (63.2%) male. The mean (+/- SEM) duration of illness was 6.04 +/- 0.29 years. In total, 234 patients (44%) had pancolitis, 121 (22.7%) had left-sided colitis and 177 (33.3%) had proctitis or proctosigmoiditis. Overall, five (0.94%) patients developed carcinoma and one (0.19%) patient had high grade dysplasia. The incidence density and risk of developing either CRC or high grade dysplasia was zero in the first 10 years of disease. In those with disease duration of 10-20 years, incidence density was 2.34 per 1000 person years' duration (PYD) for all patients with colitis and 4.5 per 1000 PYD for patients with pancolitis alone. This corresponded to risks of 2.3% and 4.4%, respectively. For those with disease duration longer than 20 years, incidence density was 2.73 per 1000 PYD for all patients and 4.9 per 1000 PYD for patients with pancolitis. This corresponded to risks of 5.8% and 10.2%, respectively. Duration of disease beyond 10 years and extent of colitis were the only risk factors significantly associated with CRC. CONCLUSIONS: The risk of developing CRC is Indian patients with UC is lower than that reported from the West. Strategies for cancer surveillance in Indian patients with UC need to be tailored accordingly.