Jejunal endocrine tumour composed of somatostatin and gastrin cells and associated with duodenal ulcer disease

Summary A case of malignant endocrine tumour of the jejunum, associated with severe duodenal ulcer is described. The tumour and a local metastasis were examined by immunohistochemistry and found to contain abundant somatostatin-immunoreactive cells together with less numerous cells displaying gastrin immunoreactivity. This is to our knowledge the first case of intestinal somatostatinoma. The presence of gastrin cells in the tumour may explain the ulcer diathesis.     

von Recklinghausen's disease associated with somatostatin-rich duodenal carcinoid (somatostatinoma), medullary thyroid carcinoma and diffuse adrenal medullary hyperplasia.

This report describes the concomitant occurrence of a somatostatin-rich duodenal carcinoid, a medullary thyroid carcinoma and a diffuse adrenal medullary hyperplasia in a patient with von Recklinghausen's disease. A 50-year-old Japanese man died from lung metastasis of a malignant schwannoma. In addition to extensive viscero-cutaneous neurofibromatosis, two different types of neuroendocrine tumors were found in the duodenum and thyroid gland at autopsy. The duodenal tumor, which was located in the second portion, showed the histologic appearance of a carcinoid tumor with glandular differentiation and psammoma-bodies. Immunohistochemically the tumor cells were intensely positive for somatostatin. The thyroid tumor was composed of nests of tumor cells arranged in an endocrine pattern, and showed immunoreactivity for calcitonin. A review of the literature revealed no previously reported case of concomitant occurrence of duodenal somatostatinoma and medullary thyroid carcinoma in a single patient with von Recklinghausen's disease. Morphometric analysis of adrenal glands disclosed the presence of diffuse medullary hyperplasia. Thus, the present case exhibited a similarity in some respects with multiple endocrine neoplasia (MEN) syndrome, Type IIa or IIb.     

Spontaneous loss of ph chromosome with maintenance of clonal hemopoiesis in an untreated patient with myeloproliferative disease and a long survival

The unusual case of myeloproliferative disease described here is characterized by the following features: (1) a clinically completely silent course for 11 years without splenomegaly, marrow fibrosis, or cellular morphologic alterations; (2) the presence, at the onset, of a Philadelphia (Ph) chromosome without DNA breakpoints in the M-bcr region; (3) the spontaneous loss of detectable Ph-positive cells, 5 years after the first finding of leukocytosis, in the absence of any therapy; (4) the maintenance of the clonal nature of hematopoiesis, as revealed by the PGK X-linked inactivation pattern, in the absence of the Ph chromosome; and (5) a biphasic trend in the levels of leukocytes, red cells, and platelets during the years of observation.     

Major karyotypic abnormality in a child born to a woman with untreated malignant melanoma

The karyotype of a 7-month-old child had 46 chromosomes, including five abnormal chromosomes in cultured lymphocytes. G-banding indicated the presence of reciprocal translocation products between chromosomes 1 and 7 and between chromosomes 4 and 15. A probable third translocation involved the same chromosome 4p arm and 12q. All meta-phases showed these changes. C-band markers and the presence of reciprocal exchange products indicated that the chromosome changes occurred in the zygote or a post-zygotic cell of the child. The mother developed malignant melanoma while carrying the child but did not receive therapy before its birth. The suggestion is made that an undetected common agent was involved in the aetiology of the mother's tumour and the clastogenic change to the child's chromosomes.     

Duodenal Carcinoid (Somatostatinoma) Cornbined with Von Recklinghausen's Disease: A Case Report and Review of the Literature

We report the immunohistochemical and ultrastructural features of a case of duodenal carcinoid (somatostatinoma) combined with cutaneous type von Recklinghausen's disease in a 65-year-old woman. The duodenal tumor located at the 2nd portion was composed of tumor cells arranged in a trabecular, glandular or nest-like pattern, occasionally associated with eosinophilic globules in the glandular structures. The tumor cells mostly showed strong immuno-reactivity with anti somatostatin antibody. Electron microscopy revealed that every tumor cell contained intracytoplasmic granules with electron-dense cores, 100–300 nm in size, in varying numbers, and intracytoplasmic lumina were rarely detected. The present case including identical cases reported so far suggest that the disease entity is likely to be a combination of duodenal somatostatinoma and von Recklinghausen's disease and/or phaeochromocytoma. This is the tenth case of duodenal carcinoid associated with von Recklinghausen's disease and/or phaeochromocytoma in the world literature and the third case reported as duodenal somatostatinoma.     

Two cases of early-stage esophageal malignant melanoma with long-term survival

Herein is described two patients with early-stage primary malignant melanoma of the esophagus with long-term survival who were treated with esophagectomy. Both tumors had similar pathological findings, and were mainly at the stage of radial growth phase. Widespread melanoses were present in the mucosa surrounding the tumors in both cases. The two patients recovered uneventfully after surgery and were in remission at follow up of 33 months and 53 months.     

Chromosome number correlates with survival in patients with malignant pleural mesothelioma.

Modal (MO) and mean (ME) chromosome numbers determined by cytogenetic analysis were compared with survival in 34 patients with diffuse malignant pleural mesothelioma. The patients with normal chromosome number and no clonal abnormalities (MO = 46) survived longer (median survival 17 months) than patients with clonal abnormalities. MO greater than 46 correlated with shorter survival (median 12 months) (p = 0.0186). The correlation was more clear between mean chromosome number (ME) and survival: the median survival of patients with ME greater than 46 was 13 months whereas that of the patients with ME less than 46 was 26 months and ME = 46 (normal chromosome number and no clonal abnormalities) 31 months (p = 0.0007). Furthermore, there was a tendency of an association between ME = 46, ME less than 46 and epithelial subtype reported to be associated with a favorable prognosis in mesothelioma. The addition of chromosome material may present a mechanism to enhance expression of genes important in the pathogenesis of mesothelioma and lead to more aggressive behavior of the tumor.     

MIB-1 proliferation index correlates with survival in pleural malignant mesothelioma

AIMS: The purpose of this study was to establish whether the cell proliferation index assessed by the monoclonal antibody MIB-1 would correlate with survival in pleural malignant mesothelioma. METHODS AND RESULTS: We studied a series of seven long-term survivors with pleural malignant mesothelioma and a group of control cases with short-term survival. All cases showed MIB-1 positive cells, and labelling indices were expressed as percentage of cells with positive nuclear immunostaining by randomly counting 1000 tumour cells. A statistically significant difference was found between MIB-1 values in the long-term survival group and the control cases with short-term survival. CONCLUSIONS: Our results indicate that the differences in biological behaviour of malignant mesothelioma in long-term and short-term survivors may be explained in part by differences in tumour growth fraction and that proliferation index could represent an important prognostic parameter for this tumour.     

Variations in the morphology of villous epithelial cells within 8 mm of untreated duodenal ulcers

In order to investigate the bio-mechanics of duodenal ulcerogenesis and compare the 'quality' of drug mediated mucosal healing, it is necessary to define the morphological appearance of ulcerative mucosae. This report describes the morphological appearance of pre-therapy, juxta-duodenal ulcer (DU) villous epithelia. Biopsies made at endoscopy from the first part of the duodenum in four healthy volunteers and 3-8 mm from the edge of the DU in 97 patients were examined by light and electron microscopy. Irrespective of whether biopsies were made from the normal or juxta-DU mucosa, the villous epithelium was populated by one, or more, of six, morphologically identifiable cell types. Control epithelia were populated with normal goblet and absorptive cells. Based on the fine-structural characteristics of the predominant cell type, pathological specimens were divided into two groups: metaplastic (Group 1) and non-metaplastic (Group 2). Group 1 specimens were either exclusively populated with fully differentiated metaplastic gastric surface mucus secreting cells (GMC) (Group 1A), or GMC in various phases of metaplastic differentiation together with abnormal goblet cells (Group 1B). Group 2 specimens were populated with 'pathological' absorptive and normal goblet cells. It is postulated that the group variations in pre-therapy juxta-DU morphology represent various phases in the natural history of duodenal ulcerogenesis and healing.     

Psychosocial variables associated with the exceptional survival of patients with advanced malignant disease

This study identified psychosocial variables associated with the exceptional survival of nine cancer patients diagnosed as terminal. During open-ended interviews, subjects described their behaviors and emotions following the onset of disease and articulated personal explanations for their survival. Despite the methodological limitations inherent in this type of research, the similarity of the subjects'' responses was compelling.     

Suppressed colony formation of peripheral blood lymphocytes in a patient with somatostatinoma

The in vitro clonal growth of T lymphocytes was examined in a patient with somatostatinoma over a period of 8 months. After phytohemagglutinin stimulation colony-forming unit T lymphocytes could not be detected as compared to healthy controls and patients with metastatic colon tumors. The somatostatin produced by this tumor was identified as somatostatin-14. Synthetic somatostatin-14 inhibits significantly the colony formation and the tritiated thymidine incorporation of human peripheral T lymphocytes obtained from healthy subjects, suggesting immunoregulative properties of this neuroendocrine peptide.     

Long term survival of an infant with Sirenomelia

We report on a 3-month-old infant whose sirenomelia was diagnosed prenatally. The infant is neurologically normal and has “fusion” of the lower limbs with associated renal dysplasia, imperforate anus, pelvic and sacral “dysplasia,” and genital abnormalities. In addition she has a preauricular skin tag and rib fusion. The infant's anomalies are compatible with life and surgical separation of the lower limbs is planned. © 1993 Wiley-Liss, Inc.     

Psychosocial correlates of survival in advanced malignant disease?

Prospective studies of the general population have isolated specific social and psychological factors as independent predictors of longevity. This study assesses the ability of these factors, plus two others said to influence survival in patients with cancer, to predict survival and the time to relapse after a diagnosis of cancer. Patients with unresectable cancers (n = 204) were followed to determine the length of survival. Patients with Stage I or II melanoma or Stage II breast cancer (n = 155) were followed to determine the time to relapse. Analysis of data on these 359 patients indicates that social and psychological factors individually or in combination do not influence the length of survival or the time to relapse (P less than 0.10). The specific diagnosis (F = 2.0, P = 0.06), performance status (F = 0.66, P = 0.62), extent of disease (F = 1.12, P = 0.89), and therapy (F = 1.08, P = 0.35) were also unrelated to the psychosocial factors studied. Although these factors may contribute to the initiation of morbidity, the biology of the disease appears to predominate and to override the potential influence of life-style and psychosocial variables once the disease process is established.