Ganglioneuromatous proliferation in the gastrointestinal tract is a rare occurrence and is usually associated with specific syndrome complexes such as multiple endocrine neoplasia Type 2B or von Recklinghausen's disease. We report here a case of diffuse intestinal ganglioneuromatosis, presenting as intestinal obstruction and chronic constipation in an 11-year-old boy. Sporadic cases of intestinal ganglioneuromatosis in the absence of any systemic manifestations are a very rare cause of enteric motility disorders in childhood, and we discuss the pathological and clinical significance of this finding. Histopathological identification of this uncommon cause of a common pediatric problem is important since the condition is amenable to surgical treatment.
Multiple myeloma is a neoplastic disease of plasma cells accounting for 13 % of haematological malignancies and 2 % of all malignancies worldwide. Ascites may develop very rarely during the course of disease in multiple myeloma. We report the case of a 78 years old male with IgG lambda multiple myeloma who initially presented with plasmacytic ascites. An exhaustive review of world literature reveals 65 cases of ascites to have been reported in myeloma over a span of 62 years (1952 till date), usually developing in the course of treatment. This is the 7th case of plasmacytic ascites to have been diagnosed at initial presentation. We review the clinical features, diagnosis, prognostic significance and treatment of such cases.
Pituitary adenoma is one of the most common intracranial neoplasms, usually presenting with endocrinopathies or visual field defects. Granulomatous hypophysitis, one of the subtypes of primary hypophysitis, is a rare neurological entity presenting as a sellar lesion with mass effect symptoms and endocrinological dysfunction. Majority of cases of primary hypophysitis are misdiagnosed as pituitary adenomas preoperatively, and histopathology is required for a definitive diagnosis. Granulomatous hypophysitis can be primary/idiopathic or secondary to a variety of causes such as infection, foreign body reactions, and systemic inflammatory conditions. The presence of tumoral microgranulomas in pituitary adenoma is exceedingly rare, having been documented only twice previously. We present a unique case of recurrent pituitary macroadenoma in a 59-year-old woman with histopathological features of idiopathic intratumoral noncaseating granulomatous inflammation. The presence of a granulomatous response in the absence of a systemic infectious or inflammatory disorder is suggestive of a local tumoral response to an as-yet unidentified antigen.
We report a case of an 8-month-old, asymptomatic child who was incidentally detected to have two cystic structures in the abdomen. Surgical exploration revealed a gastric and pancreatic duplication cyst along with a blind-ending duplication of the right ureter. Excision of the duplications was relatively straightforward, and the child made an uneventful recovery. This constellation of duplications has not been reported before.
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