Constantin Reinus

Chorangiocarcinoma : A Case Report and Review of the Literature

Chorangiocarcinoma is the name designated to a chorangioma with trophoblastic proliferation manifesting increased proliferative activity. Only 3 such cases have been published so far. Other studies challenged this entity by demonstrating that proliferation of the trophoblast around chorangioma is a common phenomenon. We present a case of a unique vascular lesion in a term placenta with a malignant trophoblastic component. Microscopic examination of a well-demarcated placental mass revealed a chorangioma with multiple nodules composed of pleomorphic cells displaying focal multinucleation, large areas of necrosis, and high mitotic activity. Immunohistochemical stains of these cells were strongly positive for pancytokeratin and the beta subunit of human chorionic gonadotropin and focally positive for HSD3B1. There was no invasion of the basement membrane, and no free-floating tumor cells in the intervillous space. No evidence of metastasis was found on follow-up of the mother and newborn. It is concluded that the tumor presented herein, displaying a histologically unequivocal malignant trophoblastic component in a benign chorangioma, is a true chorangiocarcinoma, and should be included within the category of gestational neoplasia as a tumor closely related to choriocarcinoma.

Relative expression and localization of the insulin-like growth factor system components in the fetal, child and adult intestine.

Background: The insulin-like growth factors (IGFs) are important in the development and maintenance of the gastrointestinal tract. Objectives: To compare the expression of IGFs and their receptors in the stomach and duodenum of the fetus, the child and the adult. To identify the cells mainly responsible for the production of the members of the IGF system. Methods: Tissue was obtained from fetus after abortion and from children and adults during diagnostic endoscopy and biopsy. The expression of the IGFs and their receptors was estimated by an RNAse protection assay and sections were stained with antisera to the components of IGF system. Results: The tissues from the stomach and the duodenum expressed the two IGFs and their receptors at all stages of life. The fetal IGF receptors I and II, were approximately ten times higher than in the child and IGF-II was five times higher. Immunohistochemical staining showed the components of the IGF system to be localized to the gastric glands and to the basotlateral border of the gastric epithelial cells. In the duodenum, they were concentrated at the apical portion of the epithelial tissue. They could also be identified in ganglion cells and nerves. Conclusions: The IGFs and their receptors in the stomach and duodenum are expressed in all age groups and mostly are highest in the fetus. The IGF system proteins were located in the gastric glands and epithelium and in the apical portion of the villous epithelium of the duodenum.

Amyloidosis and Gastric Bleeding in a Patient With Gaucher Disease

Goals To describe the clinical course of a patient with Gaucher disease who subsequently developed amyloidosis. Background We present a case of a splenectomized patient with Gaucher disease who developed portal hypertension secondary to an enlarged, cirrhotic-like liver, and recurrent life-threatening upper gastrointestinal bleeding. Study Despite repeated diagnostic biopsies, amyloidosis was only ascertained after death. Results Albeit very rare, there are four other similar cases in the literature, but unlike these previous reports of concurrence of Gaucher disease and amyloidosis, in this patient the gastrointestinal symptoms were life-threatening but there was no evidence of gammopathy or renal disease. Also, this is the first patient who was treated with enzyme replacement therapy for 5 years prior to manifestation of amyloidosis. Conclusions Coexistence of apparently unrelated diseases with Gaucher disease demands a greater awareness of abnormalities at the biochemical and/or molecular level to adequately manage patients with Gaucher disease, regardless of concurrent enzyme replacement therapy.

Primary Amelanotic Melanoma of the Vagina

Background: Primary malignant melanoma of the vagina is extremely rare, accounting for 0.3–0.8% of all malignant melanomas. True amelanotic vaginal melanoma showing no melanin on histological examination is exceedingly rare, accounting for only 2% of all vaginal melanomas. Case Report: We describe a 31-year-old female patient who presented with locally advanced amelanotic melanoma of the vagina, with no evidence of metastatic spread on the computerized tomography (CT) scan, but who was subsequently diagnosed as suffering from metastatic disease by positron emission tomography (PET)-CT performed a few weeks following posterior pelvic exenteration. Conclusion: Specific immunohistochemical staining with melanoma markers should be performed to confirm or exclude a diagnosis of amelanotic melanoma in all patients presenting with a vaginal mass composed of undifferentiated epithelioid malignant cells. Fluorodeoxyglucose (FDG)-PET-CT should be performed as part of the preoperative evaluation, to identify the presence or absence of metastatic disease in all patients with vaginal melanoma.

Post-chemotherapy microscopic residual prostate rhabdomyosarcoma: long-term conservative follow-up

Abstract In spite of advances in the treatment of childhood bladder and prostate rhabdomyosarcoma (RMS), the ability to detect minimal residual disease correlates imperfectly with the ultimate outcome. We report the long-term follow-up of a child with microscopic residual RMS after chemotherapy. The correct interpretation of the histologic findings spared the child unnecessary additional therapy and raises enigmatic questions about the biology of minimal residual disease.

Portal hypertension is associated with modulation of regulatory T cells

Background: Portal hypertension is a complication of liver cirrhosis. The portal vein drains the spleen and the intestines, which are both rich in inflammatory mediators. Portal hypertension- induced stress within these organs that may result in pro-inflammatory changes. The association of these changes with regulatory T cells was not addressed before. Aim: Our aim is to investigate the involvement of some subsets of regulatory T cells in portal hypertension. Methods: In the current study we used the partial portal vein ligation model to demonstrate differences in the distribution of regulatory T cells within the portal vein and the inferior vena cava associated with portal hypertension. Results: We show that CD4+CD25+FoxP3+ regulatory T cells are significantly (P <0.05) increased only in the inferior vena cava of partial portal vein ligation-rats. The development of portal hypertension was associated with the reversal of the distribution patterns in the portal vein and inferior vena cava for both CD4+ and CD8+ cells. We further show that in naïve rats CD4+IL17+ cells were significantly (P <0.05) and specifically enriched in inferior vena cava compared to the portal vein. Conclusions: These novel findings support the involvement of regulatory T cells in the inflammatory signals accompanied with acute portal hypertension.

Menetrier's disease presenting as an acute protein-losing gastroenteropathy in a 27-year-old man with Gaucher disease.

Objectives To describe a unique case of a young man with Gaucher disease who was diagnosed with Menetriers disease. Background After an acute episode of severe gastritis, the patient developed hypoalbuminemia and protein-losing gastroenteropathy, and became unwell. Study Endoscopy revealed an abnormal stomach, with rigid, thickened folds covered with viscous greyish exudates. Superficial biopsies revealed foveolar hyperplasia, acute and severe gastritis with massive inflammatory infiltrate of neutrophils in the lamina propria with pit abscess formation. Tissue cultures for Helicobacter pylori were negative. Results Snare deep particle biopsy revealed the typical features of Menetriers disease. Enzyme replacement therapy for Gaucher disease was started. Conclusion This case poses a dilemma because the patient improved spontaneously, and as such is dissimilar to other adults who develop Menetriers disease because of an infection; it is hoped that he may also not be at risk of the potential malignancies that are correlated with adult Menetriers disease. The value of enzyme treatment is considered.

Abdominal cocoon: a potential pitfall in patients with ovarian carcinoma

Background. Abdominal cocoon, or sclerosing encapsulating peritonitis, is a rare condition characterized by partial or total encasement of small bowel and mesentery by a thick fibrocollagenous sack that looks like a cocoon. Within the sack, bowel loops are drawn together causing intestinal obstruction.Case presentation. We report on a 57-year-old female patient who developed a very unusual complication of ovarian cancer: abdominal cocoon formation.Conclusions. This report highlights the need for a timely diagnosis of sclerosing encapsulating peritonitis in cancer patients.

Nerves in bone marrow of newly diagnosed therapy-related acute myeloid leukemia

![Figure][1] A 70-year-old woman, with a history of diffuse large B-cell lymphoma treated with rituximab–cyclophosphamide, doxorubicin, vincristine, and prednisone during 2014, presented in June 2016 with pancytopenia (white blood cell count, 0.6 × 109/L; hemoglobin, 9.7 g/dL; platelets,