Strategy for patients with co-existence of meningioma and intracerebral aneurysm,especially unruptured aneurysm (–seven cases and review of the literature–)

BackgroundIntracerebral aneurysms co-existing with meningiomas are rare. Treatment strategies for intracerebral aneurysms co-existing with meningiomas have not yet been established.MethodsWe studied 62 patients with intracerebral aneurysms co-existing with meningiomas in the literature including our seven cases, evaluated the various managements and outcomes, and discussed the strategy for intracerebral aneurysms, especially unruptured cases, co-existing with meningiomas. The aim of this study was to develop a guide for the management of non-subarachnoid hemorrhage (SAH) intracerebral aneurysms co-existing with meningiomas.ResultsMost intracerebral aneurysms co-existing with meningiomas are unruptured. Of course, aneurysms presenting with SAH should be treated first followed by the resection of meningiomas. In addition, intracerebral aneurysms inside or adjacent to meningiomas have a high risk of intraoperative rupture during the surgery for meningiomas, and it may be necessary to treat them first followed by the resection of meningiomas with one or two-step surgery.In nine out of 62 patients, ten intracerebral unruptured aneurysms were not treated; however, no intracerebral aneurysms ruptured during the follow-up period, and outcomes of these patients were good in eight and poor in only one.ConclusionsIntracerebral unruptured aneurysms remote from meningiomas may be treated according to the guidelines for unruptured aneurysms.In advance of microsurgery and endovascular techniques, both lesions should be treated, if possible.     

Perivascular epithelioid cell tumors of the liver misdiagnosed as hepatocellular carcinoma: Three case reports

BACKGROUNDHepatic perivascular epithelioid cell neoplasms (PEComas) are rare. Diagnostic and treatment experience with hepatic PEComa remains insufficient.CASE SUMMARYThree hepatic PEComa cases are reported in this paper: One case of primary malignant hepatic PEComa, one case of benign hepatic PEComa, and one case of hepatic PEComa with an ovarian mature cystic teratoma. During preoperative imaging and pathological assessment of intraoperative frozen samples, patients were diagnosed with hepatocellular carcinoma (HCC), while postoperative pathology and immunohistochemistry subsequently revealed hepatic PEComa. Patients with hepatic PEComa which is misdiagnosed as HCC often require a wider surgical resection. It is easy to mistake them for distant metastases of hepatic PEComa and misdiagnosed as HCC, especially when it''s combined with tumors in other organs. Three patients eventually underwent partial hepatectomy. After 1-4 years of follow-up, none of the patients experienced recurrence or metastases.CONCLUSIONA clear preoperative diagnosis of hepatic PEComa can reduce the scope of resection and prevent unnecessary injuries during surgery.     

Rheumatoid arthritis associated with transient gamma 2-heavy chain disease

Summary This paper describes the clinical history of a patient (F.-O.) with longstanding rheumatoid arthritis (RA), who subsequently developed a transient gamma 2-heavy chain disease (₂-HCD). Immunochemical studies comprised serial determinations of serum levels of intact IgG, the -HCD protein, IgA, and IgM. New applications of the rocket immunoselection and the radial immunodiffusion were used for the quantitation of the -HCD protein and intact IgG, respectively, in the presence of one another. Immunofluorescent microscopy on bone marrow cells showed cells containing -heavy chains but devoid of light chains. Protein studies of the isolated -HCD protein revealed a molecular weight of 72 000 in the dimeric form, a carbohydrate content of 9.7%, and a PCA-Val-Gln NH₂-terminal amino acid sequence. The literature on the rare coexistence of RA and -HCD in a single patient is reviewed.     

Coexistence of sarcoidosis and adult onset Still disease

Sarcoidosis is a chronic, inflammatory disease with unknown cause characterized by non-caseating granuloma formations. It can be presented with bilateral hilar lymphadenopathy, skin lesions, eye involvement and locomotor system findings. Adult onset Still disease (AOSD) is a chronic inflammatory disease which presents with fever, arthritis and typical skin rashes. The disease is rare and can be misdiagnosed due to the absence of typical clinical and laboratory findings. The association of sarcoidosis and AOSD has not been previously reported in the literature.Herein we reported the development of AOSD in a patient followed by the diagnosis of sarcoidosis. The patient did not respond to high-dose corticosteroids and methotrexate therapy, and the disease was under control with anti-IL-6 (Tocilizumab) drug.     

Nitric oxide synthase, choline acetyltransferase, catecholamine enzymes and neuropeptides and their colocalization in the anterior pelvic ganglion, the inferior mesenteric ganglion and the hypogastric nerve of the male guinea pig

By the indirect immunofluorescence method, the distribution of nitric oxide synthase (NOS)-like immunoreactivity (LI) and its possible colocalization with neuropeptide immunoreactivities, with two enzymes for the catecholamine synthesis pathway, tyrosine hydroxylase (TH) and dopamine β-hydroxylase (DBH), as well as the enzyme for the acetylcholine synthesis pathway, choline acetyltransferase (ChAT) were studied in the anterior pelvic ganglion (APG), the inferior mesenteric ganglion (IMG) and the hypogastric nerve in the male guinea pig. The analyses were performed on tissues from intact animals, as well as after compression/ligation or cut of the hypogastric nerve. In some cases the colonic nerves were also cut. Analysis of the APG showed two main neuronal cell populations, one group containing NOS localized in the caudal part of the APG and one TH-positive group lacking NOS in its cranial part. The majority of the NOS-positive neurons contained ChAT-LI. Some NOS-positive cells did not contain detectable ChAT, but all ChAT-positive cells contained NOS. NOS neurons often contained peptides, including vasoactive intestinal peptide (VIP), neuropeptide tyrosine (NPY), somatostatin (SOM) and/or calcitonin gene-related peptide (CGRP). Some NOS cells expressed DBH, but never TH. The second cell group, characterized by absence of NOS, contained TH, mostly DBH and NPY and occasionally SOM and CGRP. Some TH-positive neurons lacked DBH. In the IMG, the NOS-LI was principally in nerve fibers, which were of two types, one consisting of strongly immunoreactive, coarse, varicose fibers with a patchy distribution, the other one forming fine, varicose, weakly immunoreactive fibers with a more general distribution. In the coarse networks, NOS-LI coexisted with VIP- and DYN-LI and the fibers surrounded mainly the SOM-containing noradrenergic principal ganglion cells. A network of ChAT-positive, often NOS-containing nerve fibers, surrounded the principal neurons. Occasional neuronal cell bodies in the IMG contained both NOS- and ChAT-LI. Accumulation of NOS was observed, both caudal and cranial, to a crush of the hypogastric nerve. VIP accumulated mainly on the caudal side and often coexisted with NOS. NPY accumulated on both sides of the crush, but mainly on the cranial side, and ENK was exclusively on the cranial side. Neither peptide coexisted with NOS. Both substance P (SP) and CGRP showed the strongest accumulation on the cranial side, possibly partly colocalized with NOS. It is concluded that the APG in the male guinea-pig consists of two major complementary neuron populations, the cholinergic neurons always containing NOS and the noradrenergic neurons containing TH and DBH. Some NOS neurons lacked ChAT and could represent truly non-adrenergic, non-cholinergic neurons. In addition, there may be a small dopaminergic neuron population, that is containing TH but lacking DBH. The cholinergic NOS neurons contain varying combinations of peptides. The noradrenergic population often contained NPY and occasionally SOM and CGRP. It is suggested that NO may interact with a number of other messenger molecules to play a role both within the APG and IMG and also in the projection areas of the APG.     

Further analysis of presence of peptides in dopamine neurons

Summary Double-labeling combined with elution-restaining immunofluorescence techniques were used to analyze the extent of coexistence among the peptides cholecystokinin (CCK), peptide histidine-isoleucine (PHI)/vasoactive intestinal polypeptide (VIP), substance P and the catecholamine-synthesizing enzyme tyrosine hydroxylase in neurons of the supramammillary region and mesencephalon of the rat. Approximately 50% of the PHI/VIP-containing perikarya and about 25% of the CCK-positive cell bodies in the supramammillary region exhibited coexistence of both peptides. Only a very minor portion of these double-labeled neurons were also found to contain immunostaining for tyrosine hydroxylase (indicative of dopamine in these cells). A low percentage of the neurons contained the enzyme plus either CCK- or PHI/VIP-like immunoreactivity. A low proportion of the tyrosine hydroxylase-positive neurons in this region contained substance P-like immunoreactivity and vice versa. In other areas, small numbers of neurons in periventricular and periaqueductal regions were found to be immuno-stained for CCK, PHI/VIP and tyrosine hydroxylase. Single examples of triple-labeled (CCK-PHI/VIP-TH) somata were infrequently observed in the ventral tegmental area. These data provide further evidence of peptide/peptide and peptide/monoamine coexistence in the central nervous system. The demonstration of CCK-PHI/VIP colocalization (possibly including a minor dopaminergic component) and of substance P and tyrosine hydroxylase coexistence within neurons of the supramammillary region, which has widespread projections to many areas of the forebrain, suggests that these neuropeptides may coexist in some of these pathways and perhaps be co-released in several different regions of the brain.