Atypical carcinoid of the esophagus: a case report and review of the literature.

BACKGROUND: Although carcinoid tumors of the gastrointestinal tract are relatively common, their occurrence in the esophagus is exceedingly rare. The authors report a case of an atypical carcinoid presenting in the cervical esophagus of an 82-year-old woman. METHODS: The tumor was studied with routine, silver, and immunohistochemical techniques for cytokeratin, chromogranin, and various secretory products. In addition, the literature was reviewed for carcinoid tumors of the esophagus and the findings summarized. RESULTS: The esophageal tumor showed focal necrosis, atypical cytologic features, and increased mitotic activity. It stained diffusely for chromogranin and focally for serotonin; thus it was considered an atypical carcinoid. The patient was free of disease 9 months after excision. On review of the literature, 13 additional cases of esophageal carcinoid were found. The average age of the patients was 60 years with a male predominance of 6:1; the most common presenting symptoms included dysphagia and weight loss. The majority of tumors occurred in the submucosa of the lower esophagus, and ranged in size from 1 to 12 cm. All patients except one had surgical treatment, three received adjuvant radiotherapy or chemotherapy. Although follow-up was limited, survival correlated with stage; seven of ten Stage I or II patients were disease free whereas three of four Stage III or IV patients had died of disease; the fourth patient is alive with brain metastases. CONCLUSIONS: Esophageal carcinoid tumors are exceedingly rare neoplasms. They usually occur in the lower esophagus of males who present with dysphagia. Survival statistics are limited, but appear best correlated with disease stage.

The skeleton in congenital, generalized lipodystrophy: evaluation using whole-body radiographic surveys, magnetic resonance imaging and technetium-99m bone scintigraphy.

Congenital generalized lipodystrophy (CGL) is a rare genetic disease characterized by the absence of body fat from birth. Focal bone lesions have also been reported, but their pathophysiology is poorly understood. To characterize skeletal abnormalities further in 3 patients with CGL, we employed whole-body radiographic skeletal surveys, magnetic resonance imaging (MRI, including gadolinium enhancement), and triple phase technetium-99m methylene diphosphonate bone scintigraphy. Radiographs showed numerous focal lesions within the long bones, as described in other reports. MRI showed that the entire marrow space of the long bones was abnormal and was characterized, at least in part, by the absence of marrow fat. Prolonged T1 and T2 times and marked gadolinium enhancement were observed in radiographically normal-appearing long bone. Radiographically lytic lesions occasionally demonstrated fluid-fluid levels on MRI and enhanced peripherally after gadolinium infusion. Bone scintigraphy findings such as periarticular hyperemia were relative subtle. We conclude that the appendicular skeleton of patients with CGL is diffusely abnormal and is predisposed to focal osteolysis and cyst formation.

Case report 692: Synovial chondrometaplasia of the 1st costovertebral joint.

Synovial chondrometaplasia, also known as synovial chondromatosis, is a rare disease affecting the synovial membrane and characterized by a nodular proliferation of metaplastic cartilage. It most commonly involves large joints such as the knee, hip, and elbow, but its presence in unusual locations such as small joints and the temporomandibular joint has been described. To our knowledge, this is the first case report of synovial chondrometaplasia involving the costovertebral joint in a patient with a previous injury to that area.

Contribution of portal systemic shunt to Kupffer cell dysfunction in obstructive jaundice.

Previous animal models of biliary tract obstruction have shown that hepatic phagocytic activity is impaired secondary to Kupffer cell dysfunction. Biliary tract obstruction leads to portal hypertension and an associated portal systemic shunt. Forty-eight Sprague-Dawley rats were studied to determine the contribution of portal systemic shunt to Kupffer cell dysfunction after 21 days of obstructive jaundice or sham operation. Liver uptake of radiolabeled Escherichia coli decreased from 76.1 +/- 1.4% (sham) to 63.1 +/- 6.1% in the common duct ligation (CDL) rats (P less than 0.05); lung uptake increased from 4.0 +/- 0.6% (sham) to 20.2 +/- 4.5 (CDL) (P less than 0.05). Portal systemic shunt, determined using radioactive microspheres, increased from 2.0 +/- 1.0% (sham) to 46.6 +/- 13.1% (CDL), P less than 0.05. Although a significant portal systemic shunt does exist in this 21-day model of obstructive jaundice, it does not appear to be the only mechanism underlying Kupffer cell dysfunction.

Microvascular invasion and survival in cancer of the oral cavity and oropharynx.

Although the presence of squamous cell carcinoma within the peritumoral vascular spaces of primary lesions of the oral cavity and oropharynx has been statistically linked to regional lymph node metastases, the association of this finding with disease control and survival has not been well demonstrated. To evaluate this relationship, we have reviewed the clinical and histopathologic features of 65 consecutive cases of previously untreated T2 or greater squamous cell carcinoma of these sites managed by simultaneous surgical treatment of the primary lesion and regional lymph nodes. Of all the histopathologic features of the primary lesion studied, vascular invasion had the greatest impact on survival, as confirmed by both univariate and multivariate analysis. In addition, vascular invasion correlated statistically with local recurrence, neck recurrence, and distant metastasis.

Computed tomographic evaluation of regional lymph node involvement in cancer of the oral cavity and oropharynx.

To evaluate the efficacy of computed tomography (CT) in the identification of metastatic cervical node involvement from cancer of the oral cavity and oropharynx, 61 consecutive, previously untreated patients with T2 or greater squamous cell carcinoma of these sites were studies prospectively by CT, followed within 1 month by surgery to the primary and neck as initial treatment. The CT scan of each patient was evaluated according to the location, size, and appearance of visible nodes, and each feature was correlated with the histopathologic findings of all 83 neck specimens. A significant relationship was found between CT findings (node size, node appearance, and multiplicity of nodes) and the pathologic status of the neck using Chi-square contingency table analysis (overall chi 2 = 30.928, p less than 0.001). This data supports the role of CT in the evaluation of patients with cancer of these sites.

Pseudoangiomatous hyperplasia of mammary stroma.

Grossly circumscribed, nonhemorrhagic breast masses consisting of mammary stromal proliferations that simulated vascular lesions were studied in nine women. Histologically, a striking pattern, which appeared to consist of complex inter-anastomosing channels lined by slender spindle cells, was present in the mammary parenchyma. The importance of this benign lesion, referred to as pseudoangiomatous hyperplasia of mammary stroma, is its distinction from angiosarcoma. The patients ranged in age from 22 to 52 years; all were premenopausal. Each presented with a palpable unilateral mass, measuring up to 7 cm in diameter. The patients were treated by excisional biopsy and remained well for up to 2.5 years after excision. One patient had two local recurrences within one year of the original excision, and a second patient had a local recurrence at 14 months. No patient had another concurrent or metachronous malignant tumor of the breast or other organ, and no abnormal hormonal status was found. Complete local excision appears to be adequate treatment. It remains to be determined whether this is a neoplastic process. However, there is no evidence that it is a precursor of angiosarcoma, and ultrastructural observations demonstrate that the spaces found in the lesion are not true vascular channels. Rather, they appear to arise by a process that involves disruption and separation of stromal collagen fibers. Since small foci of this change are common in hyperplastic breast tissue from premenopausal women, it is likely that the development of a discrete tumor with this pattern represents an exaggerated form of stromal hyperplasia.

Relationship of ectopic ACTH production to tumor differentiation: a morphologic and immunohistochemical study of prostatic carcinoma with Cushing's syndrome.

A case of Cushing's syndrome in a patient with an ACTH-producing prostatic carcinoma is presented. Initial transurethral biopsy showed a moderately well-differentiated adenocarcinoma. The development of Cushing's syndrome was associated with a change in the morphology of the tumor; repeat transurethral prostatic biopsy and autopsy showed an admixture of moderately well-differentiated adenocarcinoma and anaplastic carcinoma. Immunoperoxidase studies revealed ACTH only within the anaplastic component of the tumor, which also showed strong argyrophilia. Electron microscopic studies revealed abundant dense-core granules within the anaplastic portion of the tumor with only very scanty granules in the differentiated portion. This case strongly supports the contention that cells with APUD characteristics can differentiate within epithelial tissues of multiple embryologic origins, and that the possession of APUD features does not necessarily imply origin from any specific neuroendocrine progenitor cell.