Ganglion cell like cells, diagnostic dilemma.

We report a case of cutaneous swelling found on the left anterior axillary fold of a 41-year-old man. Gross examination of specimen excised from the dermis showed a well-circumscribed nodule histologically composed of spindle cells with interspersed ganglion cell like cells. On hematoxylin and eosine (H and E) staining it was diagnosed as ganglioneuroma. Ganglioneuromas are rare, benign, fully differentiated tumors that contain mature schwann cells, ganglion cells, fibrous tissue, and nerve fibers. They are commonly found along the paravertebral sympathetic ganglia and sometimes in the adrenal medulla. However primary cutaneous ganglioneuroma is an extremely rare tumor. Immunohistochemical workup revealed a fibroblastic origin and hence the case was diagnosed as fibromatosis with ganglion cell like fibroblasts. This case report suggests that the features considered diagnostic of ganglioneuromas can occur in other cutaneous lesions and, therefore, this diagnosis cannot be offered only on the basis of H and E.

Pseudohermaphrodite presented as an irreducible inguinal hernia.

Absence of antimuellerian hormone or defective functioning of antimullerian hormone type II receptors give rise to a type of male pseudohermaphroditism known as 'persistent muellerian duct syndrome'. Such patients are phenotypically male with 46 XY karyotype and well developed muellerian duct derivatives which may be intra-abdominal or may herniate in inguinal region giving rise to a condition called as 'hernia uterine inguinale'. Cryptorchidism, transverse testicular ectopia and hypospadias are few anomalies associated with this syndrome. Though orchidopexy while leaving the uterus and fallopian tube in situ is an accepted surgical management, compromisation of blood surply to the testes or the vas may occur as vasa efferentia are embedded in the wall of uterus.

Retroperitoneal para-aortic paraganglioma.

Paragangliomas or chemodectomas are neoplasms that arise from neural crest cells and histologically resemble their adrenal counterpart, the pheochromocytoma. Seventy-one percent of the extra-adrenal paragangliomas are located in the superior or inferior para-aortic area. This tumor usually presents as an abdominal mass producing back pain. Tumor localization has improved remarkably through the use of computed tomography and I131MIBG (metaiodobenzylguanidine) scintigraphy, particularly when tumors are hormonally active. Surgical excision remains the mainstay of treatment, although vascularity can at times make excision difficult.

Placental site trophoblastic tumor.

Placental site trophoblastic tumor is very rare. It is a gestational trophoblastic neoplasm which follows normal or molar pregnancy. It is usually confined to uterus & 15-20% behave in a malignant fashion. Clinically patients present with abnormal excessive bleeding or amenorrhoea. Though several trophoblastic lesions & tumors enter in the differential diagnosis, microscopically the diagnosis is usually straightforward in hysterectomy specimens. Hysterectomy is the treatment with or without chemotherapy. Progress after surgery should be monitored by placental lactogenic hormone.

Retroperitoneal para-aortic paraganglioma.

Paragangliomas or chemodectomas are neoplasms that arise from neural crest cells and histologically resemble their adrenal counterpart, the pheochromocytoma. Seventy-one percent of the extra-adrenal paragangliomas are located in the superior or inferior paraaortic area. This tumor usually presents as an abdominal mass producing back pain. Tumor localization has improved remarkably through the use of computed tomography and I131 metaiodobenzyl-guanidine scintigraphy, particularly when tumors are hormonally active. Surgical excision remains the mainstay of treatment, although vascularity can at times make excision difficult.