Extramedullary plasmacytomas are uncommon tumors, with a worldwide annual incidence of 3 per 100,000 population. They accountfor 1% ofall tumors ofthe head and neck and 4% ofall nonepithelial tumors ofthe nasal tract. A variety of treatment options has been suggested. These treatments VQl Y according to the site ofpresentation, the presence of locoregional spread, and the histologic picture. Radiotherapy has been widely used as a treatment modality, but little has been written about surgery as a single management modality. However, such an option assumes importance in a developing nation, where patient follow-up is errati c and treatment costs must be kept low. We discuss the feasibility of surgery in such a circumstance, and we describe our surgical treatment of a case of extramedullary plasmacytoma in an elderly woman who presented to our hosp ital in India.
A case of aggressive angiomyxoma in a 25 year old woman is presented. The patient was admitted to hospital with a history of hesitancy of micturation and pain in the right iliac fossa. She was found to have a left labial mass, which was clinically diagnosed to be a Bartholin gland cyst. A pelvic ultrasound revealed an additional mass in the right paravesical region. At surgery, two distinct masses were removed, one from the right perivesical space and the other from the left labium. Both masses were rubbery, white, and gelatinous and showed similar histopathology findings of thick and thin walled vascular channels set in a loose myxoid stroma. A diagnosis of multifocal aggressive angiomyxoma was made. This is the first reported case of aggressive angiomyxoma occurring as two distinct masses in one patient (J Clin Pathol 2000;53:798-799)
Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is a rare cause of intestinal obstruction mainly affecting female neonates. We present a case of a newborn female infant with a history of abdominal distension, bilious vomiting and decreased urine output. Barium enema showed a microcolon. Patient died soon after admission and the autopsy revealed a shortened bowel, a microcolon with abundant ganglion cells in the myenteric plexus, and an enlarged urinary bladder. An interesting finding in this case was the presence of enlarged nerve bundles containing several large ganglion cells on the lateral wall of the cervix. The salient clinical and autopsy findings in this case are presented.
Intestinal neuronal dysplasia is a rare cause of bowel obstruction in neonates and infants, the diagnosis of which poses a formidable challenge to both clinicians and pathologists alike. The importance of this entity lies not only in the fact that it mimics Hirschsprung's disease, but also in that untreated cases, particularly of type A, may prove fatal. The authors describe one such case of intestinal neuronal dysplasia of type A, which was diagnosed at autopsy.
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