Amyloid goiter: first manifestation of systemic amyloidosis.

Amyloidosis is an abnormal extracellular deposit, which can occur in several tissues. The mechanism is not clearly defined. In systemic amyloidosis, all the organs can be infiltrated, but amyloid goiter as the initial manifestation of systemic amyloidosis is an exceedingly rare condition. We report a rare case of a patient who presented an amyloid goiter as the first manifestation of systemic amyloidosis. This patient had a known Crohn's disease. He developed a goiter without compressive complications. Histologic examination revealed a diffuse amyloid deposition surrounding thyroid follicles. The gland was enlarged with an eosinophilic and amorphic deposit. Confirmation of amyloid was made by the presence of congophilia and apple-green birefringence under polarized-light microscopy. An immunoreactivity was seen with AA protein. Amyloid goiter is a rare manifestation of amyloidosis. About 250 cases of amyloid goiter have been reported in the literature. The goiter enlarges rapidly and progressively, often becoming compressive like thyroid cancer. The prognosis depends on the treatment of the amyloidosis and the underlying chronic disease.

[Epithelial-myoepithelial carcinoma of the nasal cavity]. / Carcinome épithélial-myoépithélial de la cavité nasale.

OBJECTIVES: Epithelial-myoepithelial carcinoma is a rare tumor of the salivary glands with an incidence of less than 1%. Most cases arise in the parotid gland. Extraoral location is exceptional. The purpose of this study was to describe a case with nasal location, which is exceptionally reported in the literature. MATERIALS AND METHODS: We report a case of epithelial-myoepithelial carcinoma arising in the nasal cavity of a 54-year-old woman. RESULTS: The woman presented with right recurrent epistaxis and on nasal endoscopic examination was found to have a polypoid tumor in the right nasal cavity. The CT-scan demonstrated a soft tissue mass without extension to the rhinopharynx or bony destruction. Histopathologic examination revealed the tumor to consist of a mixture of a solid, tubular, and trabecular structures with a double-layered arrangement of inner eosinophilic cells and outer clear cells. Dual differentiation toward myoepithelial and epithelial cells was confirmed immunohistochemically. CONCLUSION: The occurrence of epithelial-myoepithelial carcinoma in the nasal cavity is possible. In published cases, no recurrence or metastasis has been reported in this location.

[Adenocarcinoma and gastro-intestinal stromal tumor: fortuitous association or a single carcinogenic agent? A report of 2 cases]. / Adénocarcinome et tumeur stromale digestifs: association fortuite ou mécanisme oncogène commun? A propos de deux observations.

The synchronous development of epithelial and stromal tumors of the digestive tract was rarely reported in the literature. Two such cases are described in this article. One is of a 44-year-old man presenting a high grade gastric stromal tumor associated with an adenocarcinoma of the large bowel. The other is of a 68-year-old man presenting an adenocarcinoma of the head of the pancreas associated with a low grade gastric stromal tumor. The hypothesis that the association is due to a simple coincidence particularly in areas with high rates of digestive cancer is proposed. A possibility of a genetic mutation or a single carcinogenic agent interacting with two tissues in the digestive tract and inducing the development of tumors of different histotype cannot be discarded.

[Duodenal metastatic leiomyosarcoma of the uterus. A case report]. / Métastase duodénale d'un leiomyosarcome utérin. A propos d'une observation.

The digestive metastases of uterine leiomyosarcoma are rare. We report a case of a duodenal tumor detected in a 50 year-old woman, 3 years after she underwent a total hysterectomy for uterine leiomyosarcoma. The stenosing duodenal mass was thought to be a mesenchymal tumor. A pancreaticoduodenectomy was performed. The resected lesion was morphologically similar to the uterine leiomyosarcoma. In fact, the histopathological study confirmed a submucosal tumor with typical features of the uterine leiomyosarcoma. Immunohistochemistry was performed and it showed negative CD117 and CD 34. Markers displayed positivity for actin smooth muscle and desmin. Considering the patient history and the immunohistochemical observations, we diagnosed a duodenal metastasis of uterine leiomysarcoma. Through this exceptional observation we want to emphasize the epidemiological and the pathological features of the metastatic uterine leiomyosarcoma. We will also point out the progress of tumoral cells and the histopathological distinctive criteria with a primitive digestive mesenchymal tumor.