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.

Familial calcific band-shaped keratopathy: report of two new cases with early recurrence.

We report two siblings with the rare entity of familial calcific band-shaped keratopathy (BSK). Detailed ophthalmic and systemic investigations failed to reveal any underlying causative pathology. Topical disodium ethylenediamine-tetraacetate (EDTA) was applied for 30 min to all four eyes. In addition the right eye of the younger sibling required a superficial keratectomy. An improvement in corneal clarity was seen in the immediate postoperative period in both siblings. Histopathology of the keratectomy specimen revealed linear extracellular sub-epithelial granular calcium deposits. However, an early recurrence was noted in all four eyes at four weeks postoperatively. We report the second instance in the English literature of this entity. Band-shaped keratopathy presenting without an obvious etiology merits a complete systemic and ophthalmic workup. Patients with familial idiopathic BSK could be cases with poor prognosis for treatment with EDTA due to an early recurrence of the disease.

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.

Testicular tumour with features of sex cord tumor with annular tubules associated with cryptorchidism and infertility--a case report.

Sex cord tumor with annular tubules (SCTAT) is a rare tumor seen in the ovary usually associated with Peutz-Jeghers syndrome. Testicular SCTAT are more infrequent and only four such cases have been reported in the literature. A 28-year-male presented with infertiliy. He had a history of orchiopexy 10 years back for right-sided inguinal testes. A right testicular biopsy done to investigate the cause of infertility revealed testicular atrophy with a focus suspicious of SCTAT. The right-sided orchiectomy revealed two firm nodules of 0.5 and 0.2 cms of SCTAT and sertoli cell nodule (SCN) respectively on microscopy. He did not have any features of Peutz-Jeghers or any other dysgenetic syndrome. He is asymptomatic after follow up of 26 months. This is the fifth case of testicular SCTAT and the first with SCN.

Acute fulminant visceral leishmaniasis in children--a report of two cases.

Kala-azar usually presents in older children and young adults with insidious onset of fever, splenomegaly and pancytopenia. Characteristic L.D. bodies in bone marrow or splenic aspirates are diagnostic of kala-azar. We report two cases of visceral leishmaniasis in children-1 1/2 and 10 year old with unusual presentation and fulminant course. In case 1 a female presented with fever, jaundice and bleeding manifestations. Peripheral smear revealed L.D. bodies in neutrophils as well as monocytes. The liver function tests were deranged. The child died within three days due to respiratory arrest. Case 2 was a boy who presented with fever and altered sensorium with deranged liver function tests. The patient expired within three days due to hepatic encephalopathy. Thus, it is important to consider the diagnosis of Kala-azar even when the presenting complaints are atypical and institute diagnostic and therapeutic measures early to prevent mortality.

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.

Congenital cystic adenomatoid malformation of lung--report of three cases.

Congenital cystic adenomatoid malformation (CCAM) of the lung is a rare anomaly of fetal development of terminal respiratory structures. We report three cases of congenital cystic adenomatoid malformation (CCAM) of lung Stocker's type I. All the cases presented with progressive neonatal respiratory distress. One case developed hyaline membrane because of ventilator therapy. No other congenital malformation was found in any of our cases. Two of our cases were suspected on antenatal ultrasonography. Routine prenatal ultrasonography has increased the frequency of prenatal diagnosis of congenital cystic lung malformation including CCAM. The pathogenesis, management and prognosis of CCAM are discussed along with a review of literature.

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.