Reticulum cell neoplasms of lymph nodes: a clinicopathologic study of 11 cases with recognition of a new subtype derived from fibroblastic reticular cells

Lymph nodes contain nonlymphoid accessory cells including follicular dendritic cells (FDCs), interdigitating dendritic cells (IDCs) and fibroblastic reticular cells (FBRCs). Neoplasms derived from FDCs are uncommon, and those of IDC origin are even more rare. We report the clinicopathologic features of 11 reticulum cell neoplasms, including 2 of FBRC origin. There were seven male patients and four female patients ranging in age from 13 to 73 years. All cases involved lymph nodes (cervical or supraclavicular-6 cases), (abdominal--2 cases), epitrochlear (1 case); two had more than one site of involvement (cervical lymph node and mediastinum--1 case, cervical and abdominal lymph nodes--1 case). One case of FDC tumor had concomitant Castleman's disease, plasma cell variant. Each neoplasm showed similar histology with oval-to-spindle-shaped cells in a storiform or fascicular pattern. Based on immunophenotypic findings, the neoplasms were classified as FDC (five cases), IDC (two cases), FBRC (three cases), and reticulum cell neoplasm, not otherwise specified (one case). The FDC tumors showed immunoreactivity for CD21 or CD35, vimentin, and CD68. The IDC tumors showed strong positivity for S-100 protein and variable positivity for CD68 and CD1a. The cases derived from FBRCs were positive for vimentin, desmin, and smooth-muscle actin. The neoplasm classified as reticulum cell neoplasm, not otherwise specified had similar morphologic features but showed only equivocal positivity for CD68 and vimentin. Follow-up was available for 9 of 11 (82%) cases with a mean of 3.5 years. Four of five patients with FDC tumors were alive with disease when last seen; the fifth is alive and well with no evidence of disease at 4-year follow-up. One patient with IDC tumor had a recurrence in a different nodal site. Two patients with FBRC tumor were disease free at follow-up of 2 years and 8 years, respectively. The patient with reticulum cell neoplasm, not otherwise specified, was alive and disease free 8 years after diagnosis.     

Pathological findings in human autoimmune lymphoproliferative syndrome

The defects in lymphocyte apoptosis that underlie the autoimmune lymphoproliferative syndrome (ALPS) are usually attributable to inherited mutations of the CD95 (Fas) gene. In this report, we present the histopathological and immunophenotypic features seen in the lymph nodes (n = 16), peripheral blood (n = 10), bone marrow (n = 2), spleen (n = 3), and liver (n = 2) from 10 patients with ALPS. Lymph nodes showed marked paracortical hyperplasia. Interfollicular areas were expanded and populated by T cell receptor-alphabeta CD3+ CD4-CD8- (double-negative, DN) T cells that were negative for CD45RO. CD45RA+ T cells were increased in all cases studied. The paracortical infiltrate was a result of both reduced apoptosis and increased proliferation, as measured by in situ detection of DNA fragmentation and staining with MIB-1, respectively. The paracortical proliferation may be extensive enough to suggest a diagnosis of malignant lymphoma. Many of the paracortical lymphocytes expressed markers associated with cytotoxicity, such as perforin, TIA-1, and CD57. CD25 was negative. In addition, most lymph nodes exhibited florid follicular hyperplasia, often with focal progressive transformation of germinal centers; in some cases, follicular involution was seen. A polyclonal plasmacytosis also was present. The spleens were markedly enlarged, more than 10 times normal size. There was expansion of both white pulp and red pulp, with increased DN T cells. DN T cells also were observed in liver biopsies exhibiting portal triaditis. In the peripheral blood, the T cells showed increased expression of HLA-DR and CD57 but not CD25. CD45RA+ T cells were increased in the four cases studied. Polyclonal B cell lymphocytosis with expansion of CD5+ B cells was a characteristic finding. Taken together, the histopathological and immunophenotypic findings, particularly in lymph nodes and peripheral blood, are sufficiently distinctive to suggest a diagnosis of ALPS. Of note, two affected family members of one proband developed lymphoma (T-cell-rich B-cell lymphoma and nodular lymphocyte predominance Hodgkin's disease, respectively).     

Protective effect of cysteine and vitamin E, Crocus sativus and Nigella sativa extracts on cisplatin-induced toxicity in rats

There are three ways to approach and resect the caudate lobe of the liver, that is; and isolated caudate lobectomy, a combined resection of the liver overlying the caudate lobe, and a transhepatic anterior approach by splitting parenchyma of the liver. We had two patients with neoplasms originating in the caudate lobe who underwent a complete caudate lobectomy. Both patients have been doing well without liver dysfunction. Although after the transhepatic anterior approach we anticipated an adverse effect from splitting the parenchyma of the liver, the postoperative course was uneventful and similar to that of the right side approach.     

Primary retroperitoneal germ cell tumours: excision via a thoracoabdominal extraperitoneal approach

BACKGROUND: Primary retroperitoneal germ cell tumours usually present as a large abdominal mass in young men. The testes are normal on examination and ultrasonography but there are usually raised serum levels of human chorionic gonadotrophin and/or alpha-fetoprotein. METHODS: Fourteen men (median age 33 years) with primary retroperitoneal germ cell tumours were treated by chemotherapy followed by surgical resection of the primary tumour and metastases via a thoracoabdominal extraperitoneal approach. RESULTS: There was minimal morbidity. The survival rate was 13 of 14 and the disease-free survival rate was 11 of 14 after a median follow-up of 15 months. CONCLUSION: The thoracoabdominal extraperitoneal approach for the removal of retroperitoneal germ cell tumours and their metastases after chemotherapy improves tumour clearance, morbidity and recovery time compared with the transperitoneal anterior approach.     

Ambulatory urodynamics

Ambulatory monitoring of lower and upper urinary tract continues to develop. The addition of electronic urine-loss measurements to indicate the exact time-related loss is an important new feature. In the near future, quantitative urine-loss measurements will become available. The additional possibility of plugging in a flowmeter completes the technique with respect to pressure-flow analysis. At present, however, ambulatory urodynamics is still confined to specialized urodynamic centers. Ambulatory urodynamic monitoring has shown that de-novo detrusor instability after a suspension operation is frequently missed as a preoperative diagnosis of detrusor overactivity. The first steps are being taken toward giving a quantitative analysis of detrusor activity during the filling phase. This justifies a more widespread use of this ambulatory monitoring.     

Corneal endothelial cell measurements in megalocornea

A pedigree of megalocornea manifested many of the characteristic features as well as some less common features of the disorder. Endothelial specular microscopy of affected patients disclosed normal endothelial cell densities and morphologic characteristics and increased total endothelial cell populations, suggesting a process of total corneal hyperplasia. In contrast, unpublished data indicate diminished densities in congenital glaucoma, a finding more consistent with corneal distention. We conclude that specular microscopy may be of value in differentiating the two disorders, although larger series are necessary for confirmation.     

Metastasis of malignant melanoma of the nasal mucosa to the small intestine

Malignant melanoma of the mucous membranes is rare and has a very poor prognosis. When localized in the nose, the tumor becomes apparent late in the course of the disease with nasal obstruction and epistaxis. Distant metastases are uncommon, but any organ of the body can be involved. A case of a malignant melanoma of the nasal mucosa with metastasis to the small intestine is presented, and the natural history of the metastatic disease is discussed.     

Intellectual deficits after transient tyrosinemia in the term neonate

An analysis was made of the obstetric history of 1,360 patients who were delivered in 1971. It involved 3,185 pregnancies. The aim of the study was to evaluate the risk of spontaneous termination of the pregnancy, worked out from its demographic aspect. Several ideas are worthy of consideration. The risk of spontaneous intra-uterine death is cumulative, sharply increased by a repetition of such cessation of pregnancy. The same patients often undergo intentional abortion repeatedly. The respective risks of spontaneous and provoked interruption of pregnancy have been studied in greater detail as a function of parity.