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Primary synovial sarcoma (SS) of kidney is very rare and difficult to diagnose. Here, we present a case of a 21-year-old female clinically diagnosed as renal cell carcinoma. Right nephrectomy specimen showed a cystic tumor in the upper pole of kidney with areas of hemorrhage and solid growth. Histologically, it showed poorly differentiated cells with hemangiopericytoma-like vascular pattern. Morphologic and immunohistochemical features were compatible with the diagnosis of poorly differentiated SS of kidney. Primary renal SS is a recently described entity. To the best of our knowledge, approximately 34 cases have been reported till date and this is the eighth documented case of poorly differentiated variant. Most of the time, poorly differentiated SS of kidney exhibits hemangiopericytoma like histology. Reverse transcriptase-polymerase chain reaction analysis to demonstrate SYT-SSX fusion gene transcript helps to confirm the diagnosis.

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A 23 year old Asian female presented with swelling of right knee joint for 5 years with history of exacerbations and remissions of symptoms. She was initially diagnosed as a case of suprapatellar bursitis based on clinical and X-ray findings. Further evaluation with higher imaging modalities was pathognomonic of lipoma arborescens. Patient underwent synovectomy and the diagnosis was confirmed histologically. We describe a histologically proven case of lipoma arborescens to highlight the imaging findings on X-ray, Ultrasound and Magnetic resonance imaging with arthroscopic correlation. The unique feature of this case report is multimodality imaging correlation with arthroscopy and histopathology findings. We have highlighted the pathognomonic imaging findings of this rare but benign intra-articular lesion and also discussed the differential diagnosis in detail.

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Iniencephaly is a rare neural tube defect characterized by extreme retroflexion of the head with the absence of neck due to spinal deformities. The important features that help us to diagnose a case of iniencephaly are occipital bone deficit leading to enlarged foramen magnum, fusion of malformed cervical and thoracic vertebrae, and upward turned face with chin continuous with chest because of the absence of neck. The differential diagnoses include anencephaly with spinal retroflexion, Klippel-Fiel syndrome, nuchal tumors such as teratoma, goiter, and lymphangioma and Jarcho-Levin syndrome. Previously many case reports on radiological features of iniencephaly are published, but there are very few articles on necropsy findings and differential diagnosis. In the present case we have discussed in detail the necropsy findings of iniencephaly clausus with special reference to differential diagnosis.

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In this case report we describe a rare tumor--Oncocytic variant of Mucoepidermoid carcinoma of the submandibular salivary gland with a review of the literature. Oncocytic metaplasia in salivary glands is a benign change that is associated with increasing age and also seen in a few salivary gland neoplasms', which include oncocytoma, Warthin's tumor, and the rare, oncocytic carcinoma. Oncocytic differentiation in mucoepidermoid carcinoma (MEC) is uncommon. Only twelve well-documented cases of oncocytic MEC have been reported previously all of which occurred in the parotid gland. To the best of our knowledge this is the first case of oncocytic mucoepidermoid carcinoma involving the submandibular salivary gland. The recognition of this entity is important, since most of the other primary oncocytic lesions of the salivary gland are benign.

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We present a case of papillary cystic low-grade adenocarcinoma of endolymphatic sac origin. These tumors are very rare and only a few cases have been reported in literature. They have a protracted clinical course causing progressive invasion and bony destruction. Radiologically, they appear as a heterogenous lobulated vascular mass, invading bone and compressing surrounding structures. Histologically, these tumors are composed of uniform population of cells, resembling normal endolymphatic sac epithelium. This causes considerable diagnostic difficulty. A strong index of suspicion along with clinical and radiological correlation is essential to arrive at a correct diagnosis. In some bilateral cases, association with von Hippel-Lindau disease has been noted. Radical mastoidectomy and temporal bone resection, which may sometimes necessitate sacrifice of cranial nerves, is the treatment of choice.

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A 48 year old female presented with extensive ulceration of the nasal septum of 8 months duration. Investigations confirmed the local nature of the disease. A biopsy revealed large zones of ischemic necrosis and abnormal lymphoid cells invading vessel walls and glandular structures. Florid squamous metaplasia, and pseudoepitheliomatous hyperplasia of mucosal epithelium mimicked squamous cell carcinoma and necrotising sialometaplasia. Immunohistochemistry and insitu hybridization confirmed the diagnosis of an EBV positive, Nasal NK/T cell lymphoma. A Pubmed/Medline search suggests that this is the first documented case from India.

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BACKGROUND: Extraskeletal myxoid chondrosarcoma is a rare tumor that usually occurs in the soft tissues of extremities. Cytologic features of chondrosarcoma arising from a rib and presenting as a breast mass were reported by Molyneux et al in 1995. However, to the best of our knowledge, the cytology of extraskeletal myxoid chondrosarcoma of the chest wall presenting as a breast mass has not been documented before. CASE: A 30-year-old female presented with left-sided chest pain and a hard lump in the breast of two months' duration. Fine needle aspiration cytology was done, and a possible diagnosis of mucinous carcinoma or mixed tumor of the breast was suggested, with advice to prepare a frozen section before undertaking a radical procedure. On imprint cytology and frozen section a diagnosis of extraskeletal myxoid chondrosarcoma was made and confirmed by histopathology and immunohistochemistry. CONCLUSION: It is rare for extraskeletal myxoid chondrosarcoma to occur in the chest wall. This problem is compounded by the fact that cells of extraskeletal myxoid chondrosarcoma which resemble epithelial cells, can very closely mimic some malignant breast tumors.

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Three cases of Kyrle's disease are reported. AR the three cases showed lesions with typical morphology and histology. In one instance the disease was present in the earlier generation. An interesting feature is the observation of a Koebner- like phenomenon.