Heterotopic brain tissue is an extremely rare developmental malformation. It is considered to be one of the very rare choristomatous lesions involving the oral cavity. We present a case of glial choristoma located on the dorsal surface of the tongue, midline area, in an 8-month-old African-American female baby. The clinical, histologic, and immunohistochemical features are presented. In addition, a review of previously reported cases and their probable embryogenesis are analyzed. ( info) |
2/1954. Unusual location of an ovary: ultrasonographic features and surgical correlation. We present a case of ectopic ovary in a 5-month-old baby presenting a firm nodule in the left labia majora. ultrasonography was performed and revealed an ectopic ovary. The ectopic ovary was surgically returned in adnexial location. We describe the typical findings of this entity. ( info) |
3/1954. Characterization of nodular neuronal heterotopia in children. Neuronal heterotopia are seen in various pathologies and are associated with intractable epilepsy. We examined brain tissue from four children with subcortical or periventricular nodular heterotopia of different aetiologies: one with severe epilepsy following focal brain trauma at 17 weeks gestation, one with hemimegalencephaly and intractable epilepsy, one with focal cortical dysplasia and intractable epilepsy, and one dysmorphic term infant with associated hydrocephalus and polymicrogyria. The connectivity of nodules was investigated using histological and carbocyanine dye (DiI) tracing techniques. DiI crystal placement adjacent to heterotopic nodules revealed numerous DiI-labelled fibres within a 2-3 mm radius of the crystals. Although we observed labelled fibres closely surrounding nodules, the majority did not penetrate them. Placement of DiI crystals within nodules also identified a limited number of projections out of the nodules and in one case there was evidence for connectivity between adjacent nodules. The cellular and neurochemical composition of nodules was also examined using immunohistochemistry for calretinin and neuropeptide y (NPY), which are normally expressed in GABAergic cortical interneurons. Within heterotopic nodules from all cases, numerous calretinin-positive neurons were identified, along with a few cell bodies and many processes positive for NPY. Calretinin-positive neurons within nodules were less morphologically complex than those in the cortex, which may reflect incomplete differentiation into an inhibitory neuronal phenotype. There were also abnormal clusters of calretinin-positive cells in the overlying cortical plate, indicating that the migratory defect which produces heterotopic nodules also affects development of the cortex itself. Thus, heterotopic nodules consisting of multiple neuronal cell types are associated with malformation in the overlying cortical plate, and have limited connectivity with other brain regions. This abnormal development of connectivity may affect neuronal maturation and consequently the balance of excitation and inhibition in neuronal circuits, leading to their epileptogenic potential. ( info) |
4/1954. Wet umbilicus caused by pancreatic heterotopia in urachal remains. drainage from the umbilicus may be due to the presence of structures derived from the vitelline duct or urachus, granulomas, ectopic tissue, or poor hygienic practice. This paper discusses the case of a 6-month-old infant with a wet umbilicus caused by ectopic pancreatic tissue, in urachal remains. No similar cases have been found in the literature. ( info) |
5/1954. Sessile polypoid gastric heterotopia of rectum: a report of 2 cases and review of the literature. OBJECTIVE: The term heterotopia, a term derived from Greek, implies "other place." It refers to the finding of normal tissues at foreign sites. Heterotopic gastric tissue rarely involves the large bowel. We report 2 cases of this rare entity. DATA SOURCES: case reports of 2 patients with sessile polypoid lesion in the rectum, with a review of the available literature, using both medline and relevant bibliographies of published articles. RESULTS: Biopsies of the rectal lesions in our 2 cases revealed the presence of gastric tissue. Only 27 other such cases have been reported in the English literature. CONCLUSIONS: patients with rectal gastric heterotopia usually present with bleeding, but other presentations and complications are possible. Endoscopic or surgical excision is the treatment of choice, although the lesions also respond to histamine 2 receptor blockers. ( info) |
We describe a patient with Chandler's syndrome variant of the iridocorneal endothelial syndrome in whom ectopic Descemet's membrane was found intraoperatively on the anterior surface of the lens. Initially, the membrane was confused with the anterior lens capsule during extracapsular cataract extraction, leading to the performance of a pseudocapsulorrhexis. Electron microscopy disclosed that the epilenticular membrane was composed of multiple layers of abnormal basement membrane consistent with the iridocorneal endothelial syndrome. ( info) |
7/1954. Ectopic submandibular thyroid tissue. We reported a case of a 66 year-old male patient with a mass located in the right submandibular region. Surgical excision of the mass was performed and histologic examination revealed that the tumor was ectopic thyroid tissue. Additionally, imaging studies confirmed that the thyroid gland was found in its normal location and the patient had normal thyroid function testing. In reviewing the literature, we found submandibular thyroid tissue to be a rare entity with only 4 cases described. We discussed embryologic development of the normal thyroid gland and some explanations as to the origins of several forms of ectopic thyroid tissue, including that found in the submandibular region. ( info) |
8/1954. Lingual ectopia of the thyroid gland and autotransplantation. A case of lingual ectopia of the thyroid gland in an adult patient is presented. The management by total excision and autotransplantation of the thyroid is described with a brief review of literature. ( info) |
9/1954. Primary breast carcinoma of the vulva: a case report and literature review. BACKGROUND: In 1872, Hartung was the first to describe the case of a fully formed mammary gland arising in the left labium majora of a 30-year-old woman. Since Hartung's initial report, 38 additional cases of ectopic vulvar breast tissue have been described. This case report describes the rare occurrence of primary mammary adenocarcinoma arising within the vulva. CASE: A 64-year-old G4P4 white female presented with a 4-year history of a 2 x 1 cm firm, indurated, raised lesion of the left lateral mons. A wide local excision with ipsilateral inguinofemoral lymphadenectomy was performed. Given histological findings characteristic of both invasive ductal carcinoma and invasive lobular carcinoma, in conjunction with the presence of estrogen and progesterone receptors within the tumor, a diagnosis of infiltrating adenocarcinoma arising within ectopic breast tissue was made. CONCLUSIONS: Thirty-nine reported cases of ectopic breast tissue arising within the vulva have been reported in the world literature. Though the diagnosis of primary breast carcinoma arising within the vulva is based primarily upon histologic pattern, estrogen and progesterone receptor positivity provide supporting evidence. Given the rarity of this condition, guidelines for therapy are unavailable; we therefore suggest looking to the current management of breast cancer in order to establish a sensible approach. ( info) |
10/1954. Ectopic hamartomatous thymoma. Report of a case with fine needle aspiration biopsy findings. BACKGROUND: Ectopic hamartomatous thymoma is a rare, benign tumor occurring exclusively in the supraclavicular and suprasternal regions. To the best of our knowledge, there are no English-language reports on its cytologic findings. CASE: A fine needle aspiration specimen from a mass in the suprasternal region in a 63-year-old male revealed epithelial cell nests, spindle cells, a cluster of mature adipocytes and a small number of lymphocytes. CONCLUSION: Although ectopic hamartomatous thymoma is very rare, fine needle aspiration cytology may contribute to the correct diagnosis in conjunction with the characteristic clinical findings. ( info) |