Ectopic immature renal tissue. Report of two cases

We present two cases of ectopic renal tissue. The first case had a small retroperitoneal nodule near the lower pole of the left kidney, in which the tissue was associated with nervous tissue and differentiating skeletal muscle fibers. The other case presented with a yuxtaepididimal nodule and also contained small groups of ovary hilius like cells. The possibility is raised that ectopic rests like these might be the source of extrarenal Wilms' tumours associated or not with teratomas.     

Zygomycosis in ostriches.

An unusual disease characterized by anorexia, progressive stunting and stomach impaction was observed in young ostriches after treatment with antibiotics. The massive accumulation of a clear white gelatinous mucus adherent to the inner surface of the ventriculus plus a severe necrosis of the glandular layer were the only consistent findings. Histological examination revealed typical forms of a zygomycete invading the affected tissue. The zygomycete Rhizopus oryzae was isolated from the ventriculus of all the affected ostriches.     

Liposarcoma with meningothelial-like whorls. Report of four cases showing diverse histologic findings and behavior

We report the clinicopathologic findings of four cases of liposarcoma with meningothelial-like whorls. Two cases occurred in the retroperitoneum and the remaining cases in the anterior mediastinum and scrotum. The whorls varied in terms of amount and morphology and the type tissue surrounding the whorls also varied in every case. One of the retroperitoneal cases with large areas of whorl coalescence recurred in the abdominal wall as an inflammatory malignant fibrous histiocytoma one year after primary resection of the tumor, and a metastasis to the cervical spines was detected twenty months later. The other retroperitoneal tumor recurred locally two years after the resection of the tumor and the amount and cellularity of the whorls as well as p53 reactivity and Ki-67 labeling index were higher in the recurrent tumor. However, coalescence of the whorls was not present in the recurrent tumor in contrast to the primary tumor. The anterior mediastinal and scrotal cases have demonstrated neither local recurrence nor distant metastasis although the follow-up period has been less than one year. The cells comprising whorls showed positive reactions for CD10, CD56, CD99, factor XIII, and low-affinity nerve growth factor receptor in addition to vimentin and alpha-smooth muscle actin. Our results indicate that liposarcoma with meningothelial-like whorls is a heterogeneous group that shows wide variations in histologic findings and biologic behavior. The phenotypic transformation of the whorls to higher grade in two retroperitoneal tumors, which showed recurrence within two years of follow up, supports that a whorl is a sign of dedifferentiation. Although we demonstrate the expressions of several markers, such as CD10, CD56, CD99, factor XIII, and low-affinity nerve growth factor receptor, in the spindle cells of the whorls for the first time, the lineage of the whorls still cannot be addressed due to the fact that these markers are lineage nonspecific.     

Unexpected coronary artery findings in mucopolysaccharidosis. Report of four cases and literature review

IntroductionThe mucopolysaccharidosis syndromes are a group of lethal inherited disorders affecting multiple organ systems by the progressive deposition of glycosaminoglycan. Advances in treatment such as enzyme replacement and hematopoietic stem cell transplantation have significantly improved the outcome of these disorders. An in-depth understanding of the pathophysiology of heart disease in these disorders is essential since death from cardiac causes continues to be common. Epicardial coronary artery luminal narrowing from myointimal proliferation and glycosaminoglycan deposition is well described in severe mucopolysaccharidosis type I [Hurler syndrome, mucopolysaccharide IH] but poorly understood in other “non-Hurler” phenotypes of these disorders. Given the rarity of these conditions, autopsy specimens are uncommon.MethodsTissue from epicardial coronary arteries from autopsies of four patients with non-Hurler mucopolysaccharidosis (attenuated type I, type IIIA, type IIIC, and type VI) who had died after hematopoietic cell transplantation (within 1 month in three cases; after 5 years in the fourth) was examined by light microscopy.ResultsUnexpectedly, near-normal coronary arteries were observed in the patient with attenuated mucopolysaccharidosis type I, while the coronaries from patients with type IIIA, IIIC, and VI demonstrated classic histologic features of glycosaminoglycan deposition. The most severe findings were found in the MPS IIIC patient who had 5 years of full donor engraftment after transplantation.ConclusionsOur current understanding of the cardiac manifestations of the mucopolysaccharidoses fails to explain why near-normal coronary arteries may be observed when abnormalities would be most likely to be expected and, conversely, why significant histopathology is present when it would be least expected. Identification of downstream effects of glycosaminoglycan deposition may identify other metabolites or metabolic pathways that are important in the clinicopathologic manifestations of these diseases.SummaryThe mucopolysaccharidosis diseases are a group of inherited disorders affecting multiple organ systems by the progressive deposition of glycosaminoglycan. Severe coronary artery disease is well recognized in severe type I mucopolysaccharidosis (Hurler syndrome), but unexpected coronary artery disease occurs in other, “non-Hurler” mucopolysaccharidoses. Factors responsible for the development of coronary pathology in the mucopolysaccharidoses remain elusive.     

West Nile neuroinvasive disease. Report of four cases in Northern Greece, 2018

West Nile virus (WNV) is a mosquito-borne RNA flavivirus which caused several epidemics worldwide. The year 2018 was a WNV record year for Europe, including Greece, with earlier and longer transmission season with higher than the previous number of cases. It has been proposed that some simple biochemical markers may be helpful for the recognition of WNV neuroinvasive disease, its differential from other neurological infectious diseases and prognosis. We describe four cases that suffered from WNV meningitis and/or encephalitis hospitalized in 2018 in a tertiary hospital in Thessaloniki, Greece, and investigate the importance of simple biomarkers for the recognition of WNV etiology.     

Abdominopelvic sarcoma of perivascular epithelioid cells. Report of four cases in young women, one with tuberous sclerosis.

The perivascular epithelioid cell has been proposed to be the unifying proliferating cell type in a number of lesions such as angiomyolipoma, lymphangiomyomatosis, clear cell "sugar" tumor and renal capsuloma. With the exception of rare examples of angiomyolipoma, they are non-metastasizing. We report four examples of a new member of this family of perivascular epithelioid cell neoplasms that occur in abdominopelvic location and show metastatic properties. The patients, all women, were aged 19 to 41 years (mean, 32), and presented with a tumor mass involving the serosa of the ileum, uterus or pelvic cavity. Morphologically, the tumors were composed of sheets of large polygonal cells with glycogen-rich clear or eosinophilic cytoplasm and moderately pleomorphic nuclei, traversed by a delicate vasculature, mimicking clear cell carcinoma. There were areas of coagulative necrosis and occasional mitotic figures. Intracytoplasmic brown pigment was present in two cases. Spindly cells, smooth muscle and fat were absent. Lymphovascular invasion was present in all, lymph node metastasis was documented in two and metastasis to the ovary was present in one case. Two patients developed widespread metastatic disease after 10 and 28 months from diagnosis. One patient showed the clinical signs of tuberous sclerosis. In spite of the epithelial-like appearance, the tumor cells were negative for epithelial markers but were strongly positive with the melanogenesis-related marker HMB45. Another melanogenesis marker (MART-1) was positive in two cases. Other markers including S-100 protein, vimentin, muscle-specific actin, desmin and chromogranin A were negative. Thus, these tumors are not readily classifiable in the existing schema of known entities, and show overlapping morpho-phenotypic features of clear cell "sugar" tumor of the lung and epithelioid angiomyolipoma. We consider them as sarcomas composed of a pure population of uncommitted perivascular epithelioid cell, that lack modulation toward smooth muscle or adipose cells.     

Multilobated B-cell lymphoma, a variant of centroblastic lymphoma. Report of four cases

Four cases of multilobated B-cell lymphoma, one follicular and three diffuse, are described. Many of the lymphoma cells show marked lobulation of the nuclei, and possess multiple prominent nucleoli. There are admixed classical centrocytes, classical centroblasts, and cells with morphology intermediate between classical centroblasts and multilobated cells. Multilobated cells are also observed in small numbers in germinal centres of lymph nodes showing reactive follicular hyperplasia. We believe that the multilobated B-cell may represent one form of centroblast during transition between the centroblastic and centrocytic stages. Multilobated B-cell lymphoma may be its neoplastic counterpart in which the nuclear lobulation is further exaggerated.     

Minocycline as a cause of drug-induced autoimmune hepatitis. Report of four cases and comparison with autoimmune hepatitis

We describe the clinical and liver biopsy morphologic features for 4 patients with minocycline-induced autoimmune hepatitis (group 1). We compared the serum laboratory values and liver biopsy findings from group 1 with those from 10 patients with sporadic autoimmune hepatitis (group 2). All patients in group 1 had positive serum antinuclear antibody titers, but none had positive serum anti-smooth muscle antibody titers. The morphologic findings of group 1 biopsies were those of autoimmune hepatitis in all 4 patients. In addition, 1 of these biopsy specimens also had scattered single eosinophils, unlike autoimmune hepatitis. The mean histologic activity index scores for patients in groups 1 and 2, respectively, were 6.7 and 5.4. No patients in group 1 had marked bridging fibrosis or cirrhosis, compared with 4 of 10 patients in group 2. Minocycline-induced autoimmune hepatitis is usually identical to sporadic autoimmune hepatitis. The absence of eosinophils does not exclude the possibility of a minocycline cause. In the absence of clinical or morphologic differences, a recent ingestion of minocycline should be excluded before the diagnosis of sporadic autoimmune hepatitis is established. Whether the drug is unmasking latent autoimmune hepatitis is unclear.     

Non-tropical pyomyositis in adults: Report of four cases and literature review

Four cases of non-tropical pyomyositis in adults are presented and 72 cases reported in the literature are reviewed. The diagnosis is often overlooked or delayed because most physicians are not familiar with the entity. Local signs of inflammation, fever, leukocytosis and an elevated erythrocyte sedimentation rate are common features.Staphylococcus aureus is the most common pathogen and the thigh muscles are the most common site involved. Computerized tomography is used to establish the diagnosis and surgical incision and drainage in combination with antibiotic therapy is successful in the majority of the cases.     

Metastatic squamous-cell carcinoma in pericardial effusion: Report of four cases,two with cardiac tamponade

For reasons unknown, metastatic squamous-cell carcinoma is a rare cause of pleural effusions and is even less common in pericardial effusions. A review of all pericardial effusions examined in the Cytology Service at Montefiore Medical Center over a 15-year (1980–1994) period was undertaken (N = 251). Four cases with metastatic squamous-cell carcinoma were identified among 39 malignant effusions. Two patients with metastatic squamous-cell carcinoma presented with cardiac tamponade, and the other two cases had progressive cardiac failure. The diagnostic cells on cytology evaluation were scant in all four cases but exhibited classical features of metastatic squamous carcinoma, such as cytoplasmic keratinization, intercellular bridges, and occasional “pearl” formation. Pericardial biopsies available in three patients, two with cardiac failure and one with cardiac tamponade, were negative. In all four cases the primary tumor was a bronchogenic carcinoma. Metastatic squamous-cell carcinoma is an uncommon cause of pericardial effusion and usually indicates the presence of a bronchogenic carcinoma with a rapidly fatal outcome. Cytologic examination of pericardial fluid is essential in the evaluation of such patients. Diagn. Cytopathol. 1998;18:422–424. © 1998 Wiley-Liss, Inc.     

The role of astrocytes in the formation of cartilage in gliomas. An immunohistochemical study of four cases.

The occasional presence of focal cartilage in gliomas is generally attributed to metaplasia of the mesenchymal supportive elements. While this mechanism undoubtedly exists, the present report describes a different mode of development of cartilage in four gliomas occurring in young individuals. Two of the tumors were pontine astrocytomas, one was a mixed ependymoma and astrocytoma involving the fourth ventricle and the brainstem, and one was an extraspinal malignant astrocytoma in the lumbar region of a young boy who earlier had been diagnosed as having a pontine glioma for which he received radiation treatment. In all four tumors, transitions from astrocytic to cartilaginous elements were seen, characterized by an increasing deposition of chondroid ground substance between the astrocytes and a gradual morphologic changes of the glial cells to more rounded forms with a vacuolated cytoplasm, indistinguishable from chondrocytes of mesenchymal origin. Many of these cells retained positive staining for glial fibrillary acidic protein by the immunoperoxidase method, attesting to their astrocytic nature. The production of cartilage by neoplastic astrocytes may be related to their ability to secrete, in certain circumstances and occasionally in large amounts, basement membrane material and other forms of mucopolysaccharides, which may become condensed to form a chondroid ground substance. The process appears analogous to that of cartilage formation by epithelial cells in pleomorphic adenomas of the salivary glands.     

Report of four cases ofYersinia pseudotuberculosis septicemia and a literature review

Yersinia pseudotuberculosis is a rare cause of disease in humans, the most common manifestation being mesenteric lymphadenitis accompanied by abdominal pain and fever. A septicemic form ofYersinia pseudotuberculosis infection has been reported only rarely. It is usually seen in patients with underlying disorders such as diabetes, hepatic cirrhosis or iron overload. Fifty-four cases of septicemic infection were found in the literature. The earlier published cases are reviewed, and four cases occurring in Finland during the period February to June 1992 are reported.     

Gastric inverted hyperplastic polyp. Report of four cases and relation to gastritis cystica profunda

Gastric inverted hyperplastic polyp (IHP) is a rare type of gastric polyp, and is characterized by downward growth of the hyperplastic mucosal components into the submucosa. To the best ofour knowledge, 16 gastric IHP cases have been described in the English literature, but the pathogenesis has not been established. We report the clinical and pathological findings of four gastric IHP cases. The lesions were mainly composed of hyperplastic foveolar-type glands with focal cystic dilatation. Pyloric type glands, endocrine cells, acinic cell metaplasia, and smooth muscle bundles were also seen as components of the polyp. Two cases (cases 1 and 4) coexisted with multifocal gastritis cystica profunda (GCP) and gastric adenocarcinoma. Case 4 furthermore exhibited an intermediate form between IHP and GCP. We suggest that IHP may be GCP associated with exaggeratedly hyperplastic and metaplastic changes. In case 4, the coexisting gastric carcinoma was mainly located in the submucosa, whilst the mucosal component was minimal. Five out of twenty reported gastric IHP cases, including our cases, coexisted with gastric adenocarcinoma. These facts would lead us to further investigate the relation between gastric IHP and carcinoma.     

Chronic lymphocytic thyroiditis, thyrotoxicosis, and low radioactive iodine uptake. Report of four cases.

To characterize four patients with thyrotoxicosis and a low radioactive iodine uptake, thyroid biopsies were performed, and iodine metabolism was studied. Histologic examination showed the presence of chronic lymphocytic thyroiditis, with no features of Graves's disease, in all. Detailed studies in one patient revealed insufficient metabolism of iodine to account for the clinical and chemical features of thyrotoxicosis, which implies that release of stored hormone by the inflammatory process causes the thyrotoxic state. The thyrotoxicosis in this entity subsides spontaneously. Thus, this form of thyrotoxicosis differs from the usual form found in Graves's disease in that histologie features of Graves's disease are absent, the radioactive iodine uptake is low, and specific antithyroid therapy is contraindicated. The observations further demonstrate that the radioactive iodine uptake remains a valuable tool in the diagnosis of thyrotoxicosis and the differentiation of its various forms.     

Adenomas arising in Barrett's esophagus with adenocarcinoma. Report of three cases.

Adenocarcinoma of the esophagus is a well known complication of Barrett's esophagus, and results from a dysplasia-carcinoma sequence. This report describes 3 patients with adenomatous polyps arising in Barrett's esophagus. One patient presented with multiple sessile or pedunculated polyps giving a polyposis appearance; the other two patients had single polyps associated with distinct adenocarcinoma arising in Barrett's esophagus. Polyps consisted of adenomatous proliferation with adenocarcinoma in the 3 patients. Review of the literature identified twelve previously reported cases. These cases show that although rare, adenomas may arise in Barrett's esophagus, and are most likely premalignant lesions such as other adenomas of the gastrointestinal tract.