Pigmented plexiform neurofibroma: Distinction from a large congenital melanocytic nevus.

The substantial clinical and histologic overlap between neurotized congenital melanocytic nevi and the subset of plexiform neurofibromas with hyperpigmentation and hypertrichosis of the overlying skin (pigmented neurofibroma) has led to considerable confusion in the literature. A dark-brown, hypertrichotic plaque covered much of the right lower aspect of the trunk of a 1-year-old girl with a diffuse and plexiform neurofibroma in the same area, numerous café-au-lait macules, and intertriginous freckling. The latter findings were diagnostic of neurofibromatosis-1, which was further supported by the presence of unidentified bright objects on magnetic resonance imaging of the brain. Histologic examination of the hyperpigmented plaque revealed melanocytic hyperplasia at the dermoepidermal junction and a proliferation of rounded, pigmented melanocytes dispersed individually and in occasional small nests in the papillary dermis and scattered within underlying neurofibromatous tissue. Immunohistochemical staining with A103 (Melan-A/MART-1) and PNL2 confirmed the melanocytic differentiation of the pigmented cells, whereas glial fibrillary acidic protein and Leu-7 were detected only within plexiform areas and slender neuroid spindle cells. This case draws attention to the pigmented neurofibroma as a distinct clinicopathologic entity resulting from proliferation of melanocytes and neurosustentacular cells in the setting of neurofibromatosis-1.

Thymoma in Children: Report of Two Cases and Review of Literature.

Thymoma is an uncommon tumor of childhood. Stage of the tumor is an independent prognostic factor for survival. Surgery is the treatment of choice for stage I and stage II tumors. Chemotherapy is reserved for patients with refractory or metastatic disease. Thymomas are moderately radiosensitive. However, radiation therapy is not an attractive option for children due to its side-effects on developing organs. The authors describe 2 children with completely encapsulated thymoma who were successfully treated with surgery alone. Both patients remain free of disease 3 years after surgery. One of the patients also has nevus sebaceous. The authors also discuss the possible association between the two disease entities.

Thymoma in children: report of two cases and review of literature.

Thymoma is an uncommon tumor of childhood. Stage of the tumor is an independent prognostic factor for survival. Surgery is the treatment of choice for stage I and stage II tumors. Chemotherapy is reserved for patients with refractory or metastatic disease. Thymomas are moderately radiosensitive. However, radiation therapy is not an attractive option for children due to its side-effects on developing organs. The authors describe 2 children with completely encapsulated thymoma who were successfully treated with surgery alone. Both patients remain free of disease 3 years after surgery. One of the patients also has nevus sebaceous. The authors also discuss the possible association between the two disease entities.