Pheochromocytoma in a Child without Hypertension: A Contribution to the "Rule of 10s".

Pheochromocytoma (PCC) is a neuroendocrine tumor originating from chromaffin tissue in adrenal medulla. Its diagnosis and treatment are well defined in adults, but experience in children is limited. Children constitute only 10% of reported cases, the average age at presentation being 11 years. The most common presentation is sustained hypertension, which is absent in only 10% of children. We managed a 14-month-old female child with PCC, but she was not hypertensive. We report two unusual features, in this case, an extremely young age at presentation and a childhood case of nonhypertensive PCC contributing for "rule of 10s."

Gastric GIST or gastric schwannoma-A diagnostic dilemma in a young female.

INTRODUCTION: Gastrointestinal stromal tumor (GIST) is the commonest mesenchymal tumor of GI tract and 60-70% of it seen in the stomach, whereas Gastric schwannoma is a benign, slow growing and one of the rare neoplasms of stomach. Age distribution, clinical, radiological features and gross appearance of both tumors are similar. PRESENTATION OF CASE: We report a rare case of gastric schwannoma in a 20-year-old girl, who underwent subtotal gastrectomy with the suspicion of a GIST preoperatively but later confirmed to be gastric schwannoma postoperatively after immunohistochemical study. DISCUSSION: Accordingly, the differential diagnosis for gastric submucosal mass should be gastric schwannoma. Furthermore, Gastric schwannoma is a benign neoplasm with excellent prognosis after surgical resection, whereas 10-30% of GIST has malignant behavior. Therefore, it is important to distinguish between gastric schwannoma and GIST so as to make an accurate diagnosis for optimally guide treatment options. CONCLUSION: Due to the paucity of gastric schwannoma, the index of suspicion for this diagnosis is low. So it is important to include gastric schwannoma in the differential diagnosis when preoperative imaging studies reveal submucosal exophytic gastric mass and after resection of the tumor with a negative margin, it should be sent for immunohistochemical study for confirmation of diagnosis.

Melanotic choroid plexus carcinoma of the posterior fossa.

BACKGROUND: The pigmented or melanotic variant of choroid plexus carcinoma is very rarely encountered. CASE REPORT: We report herein a case of melanotic or pigmented choroid plexus carcinoma in the posterior fossa of an 11-year-old female. CONCLUSIONS: A histopathological study supported by immunohistochemistry helped the authors to diagnose this rare entity. How to differentiate this tumor from pigmented papillary medulloblastoma, cerebellar papillary ependymoma, and metastatic malignant melanoma is discussed.

Cytodiagnosis of angiosarcoma of breast.

A 24-year-old female presented with swelling in the left breast for one-and-a-half years associated with pain for last 4 months. During this period, she was operated twice and once again there was local recurrence, the biopsy was interpreted as benign vascular lesion. The case was diagnosed by preoperative cytology as angiosarcoma of breast, after which, she underwent modified radical mastectomy. Angiosarcoma of breast is uncommon with extremely bad prognosis. Familiarity with the clinical and pathologic features is critical in avoiding underdiagnosis and delayed treatment.