Clinico-pathological profile of 22 cases of cystic renal dysplasia.

Renal dysplasia is one of the major renal developmental anomaly characterized by abnormal structural organization and development of metanephric elements. It is usually detected antenatally or in early childhood. The kidney may be multicystic, aplastic, hypoplastic or duplex. We studied 22 cases of cystic renal dysplasia diagnosed over a period often years to identify the spectrum of morphological changes in dysplastic kidney, with special emphasis on mesenchymal changes. Clinical, radiological and gross morphologicalfeatures were noted. Microscopic features were studied in detail, including the epithelial and mesenchymal changes. Twenty-one of the 22 cases studied were children. One case was a 21-year-old adult, which is a rare age at presentation. Male to female ratio was 1.1:1. One of our patients had contra-lateral ureteric stenosis, a rare anomaly reported with renal dysplasia. Ten patients, all autopsy cases, had multi-system congenital anomalies. As cystic renal dysplasia is not a hereditary disease, it must be differentiated from polycystic kidney disease. Other differential diagnoses are cystic nephroma and cystic partially differentiated nephroblastoma. Histopathological examination is the final diagnostic tool since radiological features alone may not be sufficient to exclude other cystic renal lesions. Cartilage may not be seen in all cases of renal dysplasia. Once diagnosed, other associated anomalies should also be looked for.

Pyrexia of unknown origin with neutropenia, cervical lymphadenopathy, pneumonia and marrow hypoplasia.

A 56 years adult male presented with fever for 3 weeks with neutropenia and cervical lymphadenopathy with left sided pneumonitis. Histopathology of lymphnode was consistent with Kikuchi's Necrotizing Lymphadenitis. Kikuchi's disease is usually a self- limiting illness characterized by pyrexia, neutropenia and cervical lymphadenopathy in young women of Asian decent. This often leads to the misdiagnosis of lymphoma or tuberculosis. The notable feature here is an older male presented with severe neutropenia and pneumonia with hypoplastic marrow.

Adenomatoid odontogenic tumor - a rare cause of jaw swelling

O tumor odontogênico adenomatóide (TOA) é um tumor incomum de origem odontogênica, caracterizado histologicamente pela formação de estruturas tubulares com depósitos do tipo amilóide. A histogênese do TOA ainda é indeterminada e este tumor é frequentemente considerado mais como uma lesão hamartomatosa do que propriamente um neoplasma. O TOA tem comportamento benigno, sendo suficiente a enucleação cirúrgica conservadora ou curetagem. Neste artigo, é descrito um caso de TOA em paciente de 15 anos do sexo feminino, que exibia um edema no lado esquerdo da mandíbula com reabsorção dental. A histopatologia revelou uma variante folicular intra-óssea do tumor odontogênico adenomatóide. Uma breve revisão da literatura é também apresentada.

Insular carcinoma of thyroid--a case report.

Insular carcinoma of thyroid (ICT) is a rare tumor, which accounts for 4-6% of thyroid malignancies. ICT arising from follicular cells of thyroid shows a characteristic insular growth pattern characterized by solid nests of tumor cells separated by vessels. A 52-year-old female presented with a huge swelling in midline of the neck with retrosternal extension. The patient was euthyroid. Near total thyroidectomy was done. Grossly, the tumour was involving both the lobes and isthmus of thyroid. Microscopic examination revealed features of insular carcinoma. On immunohistochemistry, tumour cells were positive for thyroglobulin and negative for calcitonin.

Angiomyoma of the palate--a case report.

Leiomyomas are rare tumors of the palate amongst which angiomyomas are the commonest sub-type. Recurrence is rare and prognosis is excellent. Only a few case reports are available in literature. We report a case of angiomyoma of the palate presenting as an encapsulated swelling in the roof of the mouth.

Lymphangioma of the fallopian tube--a case report.

Lymphangioma of the fallopian tube is very rare. Only one such case has been reported. A 30-years-old female with lymphangioma of the left fallopian tube is described here. She presented with fever, vaginal bleeding and foul smelling discharge. A mass was felt in left fornix. On exploratory laparotomy, a left tubo-ovarian mass adherent to the surrounding structures was seen. Clinical diagnosis was septic abortion with adhesions. Left-sided partial tubectomy was performed. Histology of the left tube revealed large dilated channels containing lymph in the submucosa. Lymphocytes were also seen in some channels. Histogenesis of lymphangioma remains uncertain. Presence of adhesions in this case suggests a reactive origin. Histopathology is confirmatory for diagnosis.

Sclerosing mucoepidermoid carcinoma with eosinophilia of the thyroid--a case report.

We report a 55-year old female who presented with thyroid swelling for six months. She was clinically asymptomatic with normal thyroid functions. Thyroidectomy revealed the left lobe totally replaced by a well-circumscribed grey white homogenous tumor. Microscopically, the tumor comprised of cells arranged in adenoid pattern along with nests of cohesive intermediate cells, some also forming keratin pearls in a sclerotic and inflammatory background. The thin rim of thyroid at the periphery showed features of lymphocytic thyroiditis. No other associated malignancy was seen on thorough screening. The tumor cells were negative for thyroglobulin and calcitonin. The patient was thus diagnosed with Sclerosing mucoepidermoid carcinoma with eosinophilia, thyroid.

Congenital cervical teratoma--an unusual presentation.

We report a case of congenital benign cervical teratoma in a female child. The unusual asymptomatic nature of the tumour and its relationship with the thyroid is highlighted.

Symmetric peripheral gangrene and falciparum malaria--an interesting association.

Symmetric peripheral gangrene (SPG) is a rare syndrome in which disseminated intravascular coagulation (DIC) is the most common underlying condition. We report three cases of SPG in association with Plasmodium falciparum malaria and DIC, an association unreported so far.

Intra-abdominal lymphangioma--clinicopathological profile of a rare lesion.

Lymphangioma occurring in an intra-abdominal location is reported to be extremely rare. A series of 5 cases is presented and the differential diagnoses and diagnostic features discussed. None of the cases were diagnosed as lymphangioma clinically. It is suggested that their true incidence may be unknown due to both silent clinical course as well as lack of awareness of clinical and morphological features.

Papillary cystic neoplasm of the pancreas, diagnosed by fine needle aspiration cytology--a case report.

A case of papillary cystic neoplasm of the pancreas diagnosed by fine needle aspiration cytology is presented surgical removal of the pancreatic tumour and histological study confirmed the cytologic diagnosis. A brief review of the literature and emphasis on cytological and histological features are discussed.

Composite carcinoid carcinoma of the colon--a case report.

A composite tumour of the caecum and colon, comprising of distinct areas of classical carcinoid and of well differentiated adenocarcinoma is described. Both components were histochemically confirmed. Presence of a morphologic transition at junctional areas favour a histogenesis from bi-directional differentiation of multipotent precursor cells.

Congenital cystic eyeball.

A rare case of histopathologically proved case of congenital cystic eye in a one day old girl is described. It was an unusually large cystic mass bulging forwards stretching the upperlid. There was no rudimentary eyeball in the orbit. The cystic eye's predilection for the left eye has been pointed out for the first time in this article.

Renal dysplasia with multisystem malformation--a study of 9 cases.

Nine cases of renal dysplasia encountered in a review of necropsy material are described. All these cases had associated major and minor malformations of other systems. Three out of four cases identifying with 'prune belly' syndrome had lower urinary tract obstruction. Three cases fulfilled the criteria of Meckel's syndrome. Three of these six cases, in addition, showed overlapping features with other syndromes. Three cases of unilateral dysplasia with contralateral agenesis had associated anomalies not conforming to any definite combination. We suggest that the association of malformations of developmentally unrelated systems may be random, not sharing any feature except being incompatible with life.