Adenocarcinoma of the upper esophagus arising in ectopic gastric mucosa. Two case reports and review of the literature.

Although ectopic gastric mucosa is often mentioned as a source of upper esophageal adenocarcinoma, few such cases have actually been documented. We report two cases and offer a review of the literature. Ectopic gastric mucosa in the upper esophagus is a relatively common congenital condition, but the risk of malignant transformation is extremely low. Ectopic gastric mucosa should be distinguished from Barrett's esophagus, an acquired condition with a well-documented predilection for developing adenocarcinoma.

Exocrine pancreatic tissue in human liver: a metaplastic process?

Numerous microscopic foci of exocrine pancreatic tissue consisting of acini and small ductules were distributed throughout the liver of a 41-year-old patient with severe posthepatitic cirrhosis. The acinar cells were characterized by abundant zymogen granules on electron microscopic examination and a strong reaction with antibodies to alpha-amylase on immunoperoxidase staining. The pancreatic tissue was associated with proliferations of bile ductules within areas of fibrosis. No relationship with hepatocytes was observed. A metaplastic origin of the pancreatic tissue from the intrahepatic biliary epithelium is suggested.

Recurring phyllodes tumor in aberrant breast tissue of the vulva.

A 20-year-old nulligravida woman presented with bilateral cystic nodules of the vulva, diagnosed after simple excision as benign phyllodes tumor. Breast tissue, which also displayed fibrocystic changes, was also recognized. Follow-up at 2 months revealed no evidence of disease; however, 8 months after surgery she returned with a new mass in the vulva. This was excised and found to be recurrent phyllodes tumor. Although somewhat more cellular than the first lesions, it also was deemed to be benign based on histological features supported by flow cytometric DNA studies.

Adrenal cortical adenoma in the spinal canal of an 8-year-old girl.

An ectopic adrenal cortical adenoma containing high levels of androstenedione but without clinically detectable virilizing effects was found in the spinal intradural space of an 8-year-old girl. The tumor, which was located at the L2 level, manifested itself clinically by a short history of bilateral leg pain. It was well encapsulated; therefore, total surgical removal was accomplished. The light microscopic appearance of the tumor was typical of adenomatous adrenal cortical tissue. Ultrastructurally, it also showed characteristic features of steroid-producing tumors, including very abundant smooth endoplasmic reticulum and giant mitochondria with tubulovesicular and circular cristae. Frozen tissue analyzed by radioimmunoassay was found to contain almost 20 times the normal tissue level of androstenedione. There was no elevation of cortisol or aldosterone levels in the tumor. Postoperative magnetic resonance imaging (MRI) scan of the retroperitoneum showed no abnormalities in the patient's adrenal glands. Serum androstenedione levels were normal. We postulate that the adenoma developed from congenital ectopic rests of intraspinal adrenal tissue. Although ectopic occurrence of adrenal cortical tissue has been recorded in other areas, neither such rests nor tumors developing from them have been previously reported within the spinal canal.

Hamartoma of the scalp with ectopic meningothelial elements. A distinctive benign soft tissue lesion that may simulate angiosarcoma.

Five cases of a distinctive benign soft tissue lesion of the scalp in patients ranging from 4 months to 40 years of age are described. Clinically, the lesions appeared as solitary, subcutaneous nodules suggestive of a cystic vascular malformation or other benign condition. Histologically, however, the lesions were characterized by a monotonous, pseudoinfiltrative proliferation of cuboidal epithelioid cells arranged in clusters within the dermis and subcutaneous tissue in intimate association with vessels, adipose tissue, and other connective tissue elements. A prominent feature in all cases was the presence of areas simulating freely anastomosing vascular channels lined by round to spindle-shaped, slightly hyperchromatic epithelioid cells reminiscent of angiosarcoma. Immunohistochemically, these cells were negative for factor VIII-related antigen and Ulex europaeus lectin but were strongly positive with vimentin and epithelial membrane antigen antibodies, this latter being in keeping with the immunohistochemical profile of meningothelial cells. The meningothelial nature of these cells was supported by the electron microscopic demonstration in one case of cells with complex, interdigitating cytoplasmic processes that were joined by scattered cell junctions and contained abundant intracytoplasmic intermediate filaments. The intimate admixture of meningothelial elements with haphazardly arranged connective tissue elements sets these lesions apart from cutaneous meningiomas and warrants their designation as hamartomas with an ectopic meningothelial component.

Intracardiac ectopic thyroid.

The case of a patient with an intracardiac ectopic thyroid is reported. A lesion was found in a 25-year-old man and was diagnosed by two-dimensional echocardiography as a right intraventricular tumor. An operation was performed. Histologic and ultrastructural studies showed that the tumor was a thyroid mass. The origin of intracardiac ectopic thyroids is probably to be found in disturbances occurring early in embryogenesis.

Heterotopic smooth muscle in the choroid of two patients with cryptophthalmos.

The mode of inheritance in the complete cryptophthalmic syndrome is not clear in all cases. In two of our patients with complete cryptophthalmos, one showed a probable autosomal-recessive inheritance, while in the other patient, the mode of transmission could not be determined. Light and electron microscopic studies of the globes demonstrated heterotopic smooth-muscle tissue in the anterior part of the choroid in these two patients. Although these findings might be explained on the basis of a lack of directional growth of the embryonic ciliary muscle cells during the early embryonic period, we favor a simple anatomical explanation based on differentiation arrest of the anterior segment. During the fourth month of gestation, the growing optic cup presumably fails to advance forward normally and to differentiate. The relatively normally developing posterior segment extends posteriorly. The "stretching" backward of the ora serrata during the seventh month of gestation "drags" with it the ciliary smooth muscle that did not advance forward due to the earlier arrest. Thus, in the definite cryptophthalmic eye, the smooth-muscle tissue is localized within the anterior choroid.

Ovarian hilus cell heterotopia.

A systematic pathologic study was done of the paratubal and parametrial tissues, submitted along with distal tubal segments excised for sterilization and with uteri (with or without attached adnexa) removed for common gynecologic conditions. Heterotopic ovarian hilus cell rests were noted in 12 cases, giving an overall incidence of 0.52% (12/2299). These cell rests, confined exclusively to the endosalpinx and perisalpinx, were easily differentiated from pregnancy-related decidual nodules, Walthard rests, foci of paratubal endometriosis, and ectopic adrenal rests.

An unusual case of heterotopic pancreas of the stomach.

A 56 year old man had an unusual case of heterotopic pancreas of the stomach. The patient had two lesions. One was at the gastroesophageal junction, representing the highest location in the stomach reported for this lesion. The second lesion was in the prepyloric antrum and consisted of heterotopic pancreatic tissue as well as tissue consistent with heterotopic ampulla of Vater. A general review of heterotopic pancreas is presented.

Extrameningeal meningiomas of the infratemporal fossa: diagnosis and treatment.

A presentation of two recently treated extrameningeal meningiomas of the infratemporal fossa will be augmented by a review of the literature on this subject. These tumors are quite rare, insidious in their presentation, and, as a benign tumor, require consideration of surgical treatment which causes the least possible functional disability. The natural history of these lesions and the surgical approach which has proved most successful in our hands will be discussed in some detail.

Phakomatous choristoma of the lower eyelid. A light and ultrastructural study.

This article documents the eighth reported case (to our knowledge) of a phakomatous choristoma, a rare congenital tumor of the lower eyelid of infants. Among the eight cases, there was no instance of tumor recurrence recorded nor was there any detectable postoperative eye defect, despite incomplete resection of the tumor in two patients. While usually clinically diagnosed as a dermoid cyst, the highly characteristic histologic features of this entity should allow more frequent recognition. Ultrastructural examination in our case provided additional evidence of its lenticular origin.

Gastric heterotopia in the ileum causing hemorrhage.

Two cases of infants with heterotopic gastric mucosa in the ileum causing hemorrhage and massive bleeding per rectum are presented. Preoperative Technetium 99 m scanning was valuable as a means to the diagnosis. Successful operative treatment was achieved by segmental resection with end to end anastomosis.

Aortic body tumour with adjacent ectopic thyroid tissue in a dog.

A 12-year-old neutered male Husky dog had a neoplasm at the base of the heart which did not invade surrounding tissues. Microscopically, the neoplasm was composed of nests and sheets of polyhedral cells subdivided into lobules by trabeculae of fine fibrovascular stroma. Adjacent to the neoplasm was a rim of ectopic thyroid tissue that appeared histologically normal. The possible differential diagnoses for the neoplasm were aortic body tumour, ectopic thyroid tumour and ectopic parathyroid tumour; the ultrastructural characteristics revealed it to be an aortic body tumour.

Case report of heterotopic bone formation in metastatic carcinoma of the colon.

The case of an 83-year-old woman, who was operated on for an adenocarcinoma of the sigmoid colon and died with retroperitoneal matastases, is described. The post mortem examination showed widespread heterotopic bone formation in these metastases. Gastrointestinal cancers and their metastases are liable to calcify and ossify, and they do so more frequently than other malignant epithelial tumors. A search through the literature led to the discovery of 35 other cases of this type. The highest frequency of heterotopic bone formation occurs in cancers of the distal portion of the large intestine and in pulmonary and lymph node metastases. The results of the present case support the view that bone formation derives from the metaplasia of stromal fibroblasts into osteoblasts. The knowledge that gastrointestinal cancers can calcify and ossify has a definite diagnostic relevance for the radiologist and gastroenterologist.

Fine needle aspiration cytology of recurrent ectopic meningioma.

Fine needle aspiration was performed on a recurrent extracranial meningioma in the left pterygomaxillary and temporal fossas in a 39-year-old woman. Cytologic and electron microscopic study of the aspirate and comparison to the previously resected specimen proved the recurrent nature of the lesion. The cytologic and ultrastructural findings in meningiomas are discussed with special consideration of the differential diagnosis in extracranial sites.

Phakomatous choristoma: clinical, histopathologic, and ultrastructural findings in a 4-month-old boy.

A case of phakomatous choristoma of the lower eyelid is described. A survey of previously reported cases reveals a characteristic and consistent clinical and morphologic picture. Universally the patient presents in infancy with a rather small, firm, rubbery nodule attached to the lower edge of the inferior tarsal plate. Always present in the nasal aspect of the lower lid, the tumor is easily palpable. Recognition of this clinical picture may allow the ophthalmologist to suspect the appropriate diagnosis, but the final diagnosis should be based on the recognition of characteristic histopathology.

Ectopic cervical hamartomatous thymoma showing extensive myoid differentiation.

Ectopic 'hamartomatous' thymoma is a rare benign neoplasm. These tumours are found in the neck and are thought to be part of a spectrum of ectopic cervical thymic neoplasia. The clinical and histological features are discussed and the literature is reviewed. An attempt is made to explain in embryological terms why such lesions appear to occur more commonly on the left side.

Nesidioblastosis arising from heterotopic pancreas and presenting with hypertension. A clinical, immunohistochemical and ultrastructural study.

A rare case of nesidioblastosis in an adult arising from heterotopic pancreas and presenting with hypertension is reported. To our knowledge it is the first case to be described in literature. The pathogenic mechanisms to explain hypertension are not clear. The stimulating action of glucagon on the adrenal gland or on peripheral beta receptors could be considered as hypothetical factors.

[Peri-navel intestinal patch-like choristia (author's transl)]. / Choristia intestinale périombilicale en plaques.

The authors describe an umbilical anomaly marked by confluent erythematous and crusted plaques, spreading beyond the navel limits and histologically regarded as a choristia that is to say a displacement of intestinal tissue within the epidermis. The ultrastructural study investigated the relationship between intestinal cells and keratinocytes. The authors believe that behaviour analogies exist between ectopic intestinal cells and Paget's cells.