Signet ring stromal tumor of the ovary occurring in conjunction with brenner tumor.

OBJECTIVE: A case is presented of a single ovarian mass that had elements of both signet ring stromal tumor of the ovary and Brenner tumor. METHODS: The histologic features and the results of histocytochemical, immunohistochemical, and electron microscopic studies are presented. RESULTS: The signet ring component of the mass demonstrated features consistent with the few other cases previously reported. A positive immunohistochemical stain for the peptide hormone inhibin provides additional evidence for an ovarian stromal origin for this tumor. CONCLUSION: The tumor produced no hormonally related symptoms and has shown no evidence of recurrence. Signet ring stromal tumor of the ovary is an uncommon benign tumor that may be confused with metastatic signet ring carcinoma. The significance of the signet ring cells is not certain. Their association with Brenner tumor has not been previously described.

Occurrence, etiology, and clinical significance of extreme thrombocytosis: a study of 280 cases.

PURPOSE: To determine the etiology and to evaluate the clinical consequences of an extremely elevated platelet count. PATIENTS AND METHODS: A review of the medical records was performed on all patients encountered during a 5 1/2-year period who had at least one platelet count of 1,000 x 10(9)/L or greater. RESULTS: Of the total of 280 patients with extreme thrombocytosis (EXT), 231 (82%) had reactive thrombocytosis (RT), 38 (14%) had a myeloproliferative disorder (MPD), and 11 (4%) had cases of uncertain etiology. RT was more common than MPD in all age groups except those in the eighth decade and older. Symptoms of bleeding and/or vaso-occlusive phenomena were noted in association with EXT in 21 (56%) of the MPD patients but in only 10 (4%) of the RT patients. Treatment to lower the platelet count and/or inhibit platelet function was employed in 36 MPD patients and 23 RT patients. Eight patients with MPD and 34 with RT are known to have died, but no patient in either group is known to have died of a thrombotic or bleeding event when the platelet count was greater than or equal to 1,000 x 10(9)/L. CONCLUSIONS: Platelet counts greater than or equal to 1,000 x 10(9)/L should not be considered rare events in the general, acute-care hospital population, and usually represent a reactive phenomenon.

Lymph node classification systems in cutaneous T-cell lymphoma. Evidence for the utility of the Working Formulation of Non-Hodgkin's Lymphomas for Clinical Usage.

BACKGROUND: This study was undertaken to compare three classification schemes used to evaluate lymph nodes (LN) obtained from patients with cutaneous T-cell lymphoma (CTCL): a modified Rappaport classification, the National Cancer Institute-Veterans Administration (NCI-VA) classification based on the relative numbers of cerebriform cells in the paracortical areas, and the Dutch classification based on the presence of cerebriform cells with large nuclei in mycosis fungoides (MF) and diffuse infiltration by cerebriform cells in Sézary syndrome. METHODS: A study set of 195 LN obtained from patients with CTCL (MF, Sézary syndrome, and nonepidermotropic T-cell lymphomas) and 14 LN from patients with benign dermatoses was reviewed independently by three groups of pathologists familiar with each classification system. RESULTS: Each classification system provided useful prognostic information. However, contrary to prior reports, no significant difference in survival was apparent in patients with uneffaced LN when classified according to the NCI-VA (LN0-2 versus LN3) or Dutch (Gr0-1 versus Gr2) ratings. In addition, all classification systems demonstrated a poor survival time associated with effaced LN. By combining results from the modified Rappaport and Dutch classifications, three prognostic groups could be identified based on cell morphology: a low-grade category with a small cell histologic subtype (median survival time, 40 months); a high-grade immunoblastic subtype (median survival time, 9 months) composed of cells with an oval nucleus containing a large, usually solitary central nucleolus; and an intermediate-grade category composed of all cases without the distinctive small cell and immunoblastic morphologies (median survival time, 26 months). CONCLUSIONS: The authors propose that clearly involved LN in CTCL can be categorized on the basis of cell morphology into prognostic groups analogous to what has been proposed for the Working Formulation for Non-Hodgkin's Lymphomas for Clinical Usage.

An incidental finding of extreme self-limited thrombocytosis of unknown cause.

This report describes an 11-year-old male who presented to his pediatrician at the Bowman Gray School of Medicine in Winston-Salem, N.C., because of long standing enuresis. During evaluation and the following two-week period, this patient was found to have extreme thrombocytosis ranging from 2,175 x 10(9)/L to 3,700 x 10(9)/L. In the absence of any apparent reactive cause, a presumptive diagnosis of essential thrombocythemia was made. Although chemotherapy was considered, the patient was temporarily lost to follow-up before there was a final decision about therapy. One year later, this patient's platelet count had spontaneously decreased to normal (273 x 10(9)/L), and has remained normal during a follow-up period of seven years, although only sporadic platelet counts have been obtained. His most recent physical examination revealed no abnormalities. The case is presented as further evidence that extremely high platelet counts are not necessarily dangerous and do not all require therapy.

The frequency and significance of megakaryocytic emperipolesis in myeloproliferative and reactive states.

Sixty-three bone marrow (BM) biopsy paraffin sections from patients with platelet counts of 1000 x 10(9)/1 or greater were examined to determine the incidence of megakaryocytic emperipolesis for the various myeloproliferative disorders (MPDs) and for reactive thrombocytosis. Of those cases classified as specific MPDs, 77% of primary thrombocythemia (PT) specimens, 100% of the polycythemia vera (PV) specimens, a single idiopathic myelofibrosis (IMF) specimen, and 17% of the chronic granulocytic leukemia (CGL) specimens demonstrated emperipolesis within megakaryocytes. Two of three cases grouped as MPDs but not further classified also demonstrated emperipolesis. Of the cases of reactive thrombocytosis (RT), 75% showed the presence of emperipolesis. Our results indicate that, with the exception of CGL, emperipolesis can be found in the BM megakaryocytes of the great majority of patients who have extreme thrombocytosis. The underlying cause, whether myeloproliferative or reactive, does not apparently influence the incidence of the phenomenon.

Masculinizing sclerosing stromal tumor of the ovary during pregnancy.

Sclerosing stromal cell tumors of the ovary are an uncommon neoplasm that usually does not produce hormonal imbalances. Most patients showing a hormonal effect from this lesion have had menstrual cycle disturbances. Infertility and endometrial hyperplasia have also been described. One other reported case had masculinizing effects. Other authors have documented elevated levels of both estrogenic and androgenic hormones that corrected after surgery. A case of a pregnant 27-year-old Caucasian with hirsutism on her chin and neck and a male suprapubic hair pattern is presented. Elevated androstenedione, dehydroepiandrosterone, and free testosterone levels were present. A 3-cm left ovarian mass was excised and identified as a sclerosing stromal tumor. The histologic features included a pseudolobular pattern with focal areas of sclerosis and a two-cell population of spindled and polygonal cells. Immunohistochemical studies showed a positive vimentin reaction, weakly positive desmin and muscle-specific actin stains, and a negative cytokeratin stain. Following surgery the hormone levels returned to normal and the hirsutism resolved. A normal female infant without evidence of masculinization was delivered from the patient at term.

Glucagonoma presenting as morbid obesity.

A case is described that represents the only reported patient with glucagonoma syndrome and morbid obesity. The diagnosis of glucagonoma should be considered in any patient with the classic criteria despite weight gain. The criteria for diagnosis of glucagonoma are 1) the presence of a glucagon-secreting tumor, 2) hyperglucagonemia, and 3) the clinical manifestations of either necrolytic migratory erythema, glucose intolerance, or hypoaminoacidemia.

Urinary Charcot-Leyden crystals in the hypereosinophilic syndrome with acute renal failure.

A 48-year-old man with idiopathic hypereosinophilic syndrome (IHS) developed blast crisis along with a fulminant autoimmune hemolytic anemia. Hemoglobinuria and anuric acute renal failure (ARF) ensued. Urinalysis revealed countless Charcot-Leyden crysals (CLC). This is the only known report of Charcot-Leyden crystalluria. The CLC protein (lysophospholipase) should normally undergo glomerular filtration and catabolism by the tubules during reabsorption. Its abundant presence in the urine of our patient may reflect impairment of tubular reabsorption, saturation of the tubular reabsorptive process by excessive CLC load through residual functioning glomeruli, or a combination thereof. The extreme degree of hypereosinophilia suggests a massive load of CLC protein and acute tubular necrosis implies impaired tubular function, so both mechanisms should have been operative. At the autopsy, no eosinophilic infiltrates were present in the kidneys, which points against a local spillage of CLC protein into the tubules.

myc family DNA amplification in small cell lung cancer patients' tumors and corresponding cell lines.

Tumor specimens procured from 38 different small cell lung cancer patients were studied for DNA amplification of the myc family of protooncogenes (c-myc, N-myc, and L-myc). Six of the 38 specimens (16%) had 4-fold or greater myc family DNA amplification (N-myc in 4 and L-myc in 2). All 6 tumors with amplification came from patients who had received combination chemotherapy. The myc family gene copy number of the DNA prepared from 9 tumor cell lines established from these 38 patients was similar to the myc family gene copy number in the DNA prepared from fresh tumor specimens from these same patients. myc family DNA amplification is present in 16% of small cell lung cancer patients' tumors and the amplification pattern in the tumor cell lines is representative of the fresh tumors obtained from the same patients.

Homicide as a cause of the sudden infant death syndrome.

The homicidal asphyxiation of a 10 1/2-month-old male infant and the attempted asphyxiation of his 4-month-old sibling, documented by parental confession, is presented as evidence that murder may sometimes be mistaken as sudden infant death syndrome (SIDS). A review of the literature of the relationship between murder and SIDS deaths reveals the suspicions of some physicians but few published cases; this reflects not only the difficulties of making a determination of murder by suffocation, since no injuries may be present, but also a lack of awareness among physicians who must evaluate infant deaths. It is recommended that murder should be considered in the differential diagnosis of sudden, unexpected death in infants and that the autopsy should include full-body x-rays and at least an initial look at the social history of the child.