Chemodectoma of the orbit. Apropos of a case and review of the literature

The clinical, follow up and diagnostic features of a case of chemodectoma (paraganglioma) of the orbit are reviewed. 30 cases are reported in the literature. Chemodectoma may arise in any area of the body where non chromaffin paraganglion structures are situated. These are localized in the orbit of the chimpanzee but not in the orbit of man. Malignant behavior of chemodectoma has been a controversial subject, with most authors considering them to be benign tumors. Some authors have diagnosed locally recurrent and infiltrative tumors as malignant. The authors report one case of chemodectoma of the orbit. A 5 year old child has had right exophthalmos. She was treated by tumorectomy. The follow-up is marked by recurrence of the tumor and infiltration of the right hemi-face.     

Colonic adenocarcinoma and bilateral malignant ovarian sex cord tumor with annular tubules in Peutz-Jeghers syndrome

Peutz-Jeghers syndrome is characterized by multiple polyps throughout the gastrointestinal tract in association with mucocutaneous pigmentation. Although Peutz-Jeghers syndrome polyps are hamartomas, frequent association of this syndrome with both gastrointestinal and non-gastrointestinal tumours had led to reassessment of the cancer risk in this hereditary disorder. The most common gynaecological tumors in this syndrome are adenoma malignum of the uterine cervix and ovarian sex cord tumor, particularly sex cord tumor with annular tubules. The question of malignant change in a polyp or of the association of gastro intestinal carcinomas still discuss. The authors report a case of Peutz-Jeghers syndrome in a young patient who developed a colonic adenocarcinoma in a hamartomatous polyp together with an incidentally discovered bilateral malignant sex cord tumours. We discuss its association with certain benign and malignant tumors and the risk of rare complications of these hamartomatous polyps. Although malignant tumors are increasingly reported in association with the Peutz-Jeghers syndrome, to our knowledge, there have been no previous reports of such an association in the literature.     

Malignant granular cell tumor of the abdominal wall

The granular cell tumor is an uncommon tumor that usually appears as a solitary small nodular growth and runs a benign course. It occurs widely throughout the body, but is rarely described in the abdominal wall. The authors report a case of malignant granular cell tumor which was arising in anterior abdominal wall of a 67-year-old woman. Malignant variant is rare and the abdominal wall site is extremely uncommon. Regarding this clinical case and the literature the authors purpose to review the criteria of malignancy.     

Huge cystic lymphangioma of the adrenal gland. A case report and review of the literature

Cystic lymphangioma of the adrenal gland is a rare and benign lesion, most often found incidentally during abdominal imaging studies, abdominal surgery or at autopsy. Preoperative diagnosis is difficult. Diagnosis is histological. We report a 22-year-old female presented with abdominal pain. Laboratory findings were normal. Radiological examination revealed a 35 cm left adrenal cyst. The cyst was resected and the diagnosis of cystic lymphangioma of the adrenal gland was histologically made. In presenting this case and reviewing the literature, we seek to make better known the epidemiologic, the clinicopathologic presentation and the prognosis of this rare entity.     

Desmoplastic small round cell tumor of the abdomen. Report of two cases

Desmoplastic small round cell tumor (DSRCT) is a rare clinicopathological entity individualized in 1989. Its etiopathogenesis is still unknown, and diagnosis can be achieved only by immunohistochemistry and cytogenetic studies. The objective of this work is to report two new cases of DSRCT and to review the literature to clarify its epidemiological, clinical and pathological aspects.