Sclerosing peritubular nodules: a hereditary renal abnormality in von Recklinghausen's disease

A previously unrecognized type of renal lesion characterized by the formation of multiple hamartomatous sclerosing peritubular nodules in the cortex is described. These lesions were hereditary, appearing in three members of a family, which included the mother and her two sons, in association with familial central von Recklinghausen's disease (bilateral acoustic schwannomas). The nodules originated as concentric peritubular proliferations of collagen producing spindle cells, which ultrastructurally showed myofibroblastic differentiation. Four stages of nodule development leading to progressive sclerosis and tubular destruction are described. In spite of widespread involvement of the cortex with numerous nodules, in none of the three patients was there any clinical manifestation of renal dysfunction or hypertension.     

Palmar fibromatosis (Dupuytren's contracture)

Summary Forty three cases of palmar fibromatosis were studied by light and electron microscopy, enzyme histochemistry, and ultrastructural immunohistochemistry. By electron microscopy most of the cells composing the nodules in both the proliferative and the involutional stages were identical to myofibroblasts. The myofibroblasts in the involutional nodules often possessed microfilament aggregates probably representing contraction of micro(actin)fllaments in the cytoplasm. The proliferative nodules revealed small perivascular haemorrhages and haemosiderin deposits accompanied by accumulation of macrophages and some lymphocytes; these inflammatory cells possibly secrete a certain growth factor inducing proliferation of genetically abnormal fibroblasts and myofibroblasts. Diaminopeptidase IV was detected in myofibroblasts and fibroblasts by enzyme histochemistry and ultrastructural immunohistochemistry; the enzyme may play a role in the metabolism of intercellular substances. Some perivascular mesenchymal cells, interpreted as variants of myofibroblasts, had moderate activity of alkaline phosphatase.This work was supported by Alexander von Humboldt-Stiftung     

Epithelioid angiosarcoma arising in a deep-seated plexiform schwannoma: a case report and literature review

Angiosarcoma developing within a schwannoma is rare, and only 8 cases have been reported in the literature. Its association with a plexiform schwannoma has never been reported. We describe an epithelioid angiosarcoma arising in a plexiform schwannoma of the sciatic nerve in a 73-year-old man not known to have von Recklinghausen disease. Grossly, the tumor forms a long tubular mass with a multinodular growth pattern on the cut surface. Microscopically, these nodules display characteristics of a schwannoma, including Antoni A, Antoni B, and Verocay bodies. Adjacent to these nodules are high-grade anaplastic epithelioid cells growing in solid sheets, cords, and nests with focal vasoformative channels. The anaplastic cells are immunoreactive to CD31, CD34, and factor VIII related antigen, supporting their endothelial differentiation. Local recurrence and multiple pulmonary metastasis occurred 3 months after complete excision of the tumor. This case demonstrates the first occurrence of an epithelioid angiosarcoma in a deep-seated plexiform schwannoma with an aggressive clinical behavior.     

Synchronous duodenal neuroendocrine tumours in von Recklinghausen''s disease—a case report of co-existing gangliocytic paraganglioma and somatostatin-rich glandular carcinoid

The co-existence of a gangliocytic paraganglioma and a glandular psammomatous carcinoid in the duodenum of a patient with von Recklinghausen's disease and bilateral phaeochromocytomas is reported. The two lesions were considered to be distinctive by light microscopy, electron microscopy and immunocytochemistry. The cells of the glandular carcinoid showed strong cytoplasmic immunoreactivity for somatostatin and contained only scanty intracytoplasmic microfilaments on electron microscopy. In contrast, the endocrine cells of the gangliocytic paraganglioma were positive for pancreatic polypeptide and serotonin, were negative for somatostatin, and contained conspicuous intracytoplasmic aggregates of filaments. The histogenic relationship between the two tumours is discussed. This case strengthens the known association of glandular duodenal somatostatinoma with von Recklinghausen's disease and phaeochromocytoma and, in the light of a previous case report, suggests that von Recklinghausen's disease and gangliocytic paraganglioma may co-exist more commonly than expected.     

Neurofibroma of the gallbladder seen as a papillary polyp

A case of neurofibroma of the gallbladder in a 44-year-old woman without von Recklinghausen neurofibromatosis but with right hypochondralgia is reported. The tumor was seen preoperatively as a polypoid lesion and in the resected gallbladder as a papillary polypoid growth over the main intramural tumor mass. Microscopically, the tumor was identified as a neurofibroma with many tactile corpuscle-like structures (Wagner-Meissner bodies), which were found by electron microscopic and immunohistochemical examination to consist of Schwann cells. Twelve cases of non-epithelial neoplasms arising in the gallbladder are also reviewed.     

Ribosome-Lamella Complexes in the Perineurial Cells of Neurofibromatosis

Ribosome-lamella complexes are occasionally seen in neoplastic disorders of the hematopoietic system, particularly in hairy cell leukemia. However, these structures are rare in epithelial and mesenchymal cells. In this report three tumors from a patient with neuro-fibromatosis (von Recklinghausen's disease) are examined by electron microscopy. Ribosome-lamella complexes were seen in the perineurial cells. This may be the first report of these complexes in nerve sheath tumors.     

Multiple small intestinal stromal tumors associated with neurofibromatosis-1

Gastrointestinal stromal tumors (GISTs) are rarely noted in association with neurofibromatosis-1 (NF-1, von Recklinghausen disease) as an individual gastrointestinal manifestation. We report here a case of multiple GISTs with an abundant skeinoid fiber in the jejunum of a 43-year-old woman diagnosed as NF-1. Histologically, the tumors were composed of uniform spindle-shaped cells with a fascicular pattern, almost indistinguishable from the histology characteristic of usual GISTs. However, multiple synchronous tumor occurrence, abundant skeinoid fiber, and presence of microscopic miniatures of stromal tumors are additional characteristic features of this case.     

Structural response of adult rat sertoli cells to peritubular fibroblasts in vitro

Sertoli cells were harvested from sexually mature rats and maintained in vitro for up to ten days (Sertoli cell-enriched cultures) or co-cultured with rat peritubular fibroblasts. Cultures were examined by differential light microscopy and by scanning or transmission electron microscopy. The presence of peritubular fibroblasts in co-culture greatly enhanced plating efficiency and viability of adult Sertoli cells. Sertoli cell aggregates preferentially adhered to peritubular cells, and by ten days had flattened and spread across these cells. Sertoli cells retained their characteristic ultrastructural features. Early in co-culture a collagen-like extracellular material was seen bridging Sertoli and peritubular cells, and its appearance was coincident with the presence of swollen rough endoplasmic reticulum in peritubular cells. Interperitubular cell spaces became engorged with a fibrillar material morphologically similar to the basal lamina of the seminiferous tubule wall. In the absence of peritubular cells, in culture medium “conditioned” by prior incubation with peritubular cells but not containing them, or when cultured with other fibroblastic cells, plating efficiency was low; Sertoli cells never flattened and cell ultrastructure progressively degenerated. The results indicate that peritubular cells support adult Sertoh cells in culture, possibly via extracellular material derived from peritubular cells, and suggest that Sertoli cells have an inductive effect on peritubular cell secretory activity.     

Lipid rich rhabdomyosarcoma.

A lipid rich rhabdomyosarcoma of the paratesticular region was studied by light microscopy, histochemistry, immunohistochemistry and electron microscopy. The tumour was composed of primitive looking, vacuolated, and pleomorphic cells. Lipid was present in varying amounts in all cells but was especially abundant in the vacuolated and pleomorphic cells. Some cells showed eosinophilic fibrillary cytoplasm but cross-striations were not seen. Tumour cells were positive for desmin, muscle specific actin, and vimentin. A few cells were myoglobin positive. At electron microscopy, the presence of lipid was confirmed, while thick and thin filaments, Z disks, lamina and glycogen were observed, thereby confirming striated muscle differentiation. Although moderate amounts of lipid can be expected in almost any tumour, lipid rich rhabdomyosarcomas have received little attention. The present report provides a comprehensively examined case of such a tumour initially presenting diagnostic difficulty because of its possible confusion with liposarcoma.     

An infarct-like myocardial lesion experimentally induced in wistar rats with Africanized bee venom

There is evidence in the literature that death following a bee or wasp sting may result from cardiac involvement. This study describes acute cardiac lesions experimentally induced in Wistar rats submitted to intravenous inoculation of Africanized bee venom (ABV) and killed 1, 4, and 24 h after inoculation. Significant increases in serum enzyme levels were detected; light microscopy showed necrosis of the myocardium; and enzyme histochemistry showed inactivation of enzymes in and around the areas of necrosis. This is the first report of an acute necrotizing cardiac lesion, similar to human myocardial infarction, produced by the inoculation of ABV.     

多部位发生的上皮样血管内皮瘤2例报道及文献复习

目的 探讨多部位发生的上皮样血管内皮瘤(EHE)的临床病理学特征.方法 回顾性复习2例多部位发生的血管内皮瘤的临床资料、组织学形态和免疫学表型,并复习文献.结果 2例均为女性,年龄分别为24和37岁,CT示双肺及肝弥漫结节影.病理学检查发现肝和肺结节的镜下形态相同,即在黏液样的间质背景中可见条索样、小巢状或单个散在分布的上皮样瘤细胞,瘤细胞有明显异型性,伴胞质内管腔形成,腔内偶见红细胞.免疫组织化学染色显示:瘤细胞表达内皮标记(CD34、CD31和F8),不表达CK、CD68、ER、PR.结论 上皮样血管内皮瘤是一种少见的低度恶性血管肿瘤,多部位同时发生者容易被误诊.上皮样血管内皮瘤的临床表现和生物学行为因其发生部位不同而有较大的差异.     

Dupuytren's Contracture: Fibroblast Contraction?: An Ultrastructural Study

In 6 cases of Dupuytren's disease and 1 of Ledderhose's disease, the nodules of the palmar and plantar aponeurosis were examined by light and electron microscopy. The cells composing these nodules, presumably fibroblasts, showed three significant ultrastructural features: (1) a fibrillar system similar to that of smooth muscle cells; (2) nuclear deformations such as are found in contracted cells, the severest being recognizable by light microscopy (cross-banded nuclei); (3) cell-to-cell and cell-to-stroma attachments. Based on these data and on recent information about the biology of the fibroblasts, it is suggested that these cells are fibroblasts that have modulated into contractile cells (myofibroblasts), and that their contraction plays a role in the pathogenesis of the contracture observed clinically.     

NEUROFIBROMA OF THE GALLBLADDER SEEN AS A PAPILLARY POLYP

A case of neurofibroma of the gallbladder in a 44-year-old woman without von Recklinghausen neurofibromatosis but with right hypochondralgia is reported. The tumor was seen preoperatively as a polypoid lesion and in the resected gallbladder as a papillary polypoid growth over the main intramural tumor mass. Microscopically, the tumor was identified as a neurofibroma with many tactile corpuscle-like structures (Wagner-Meissner bodies), which were found by electron microscopic and immunohistochemical examination to consist of Schwann cells. Twelve cases of non-epithelial neoplasms arising in the gallbladder are also reviewed. ACTA PATHOL JPN 38: 259–268, 1988.     

Gamna Gandy bodies: Features mimicking a mycotic disease. Two cases in the spleen

We report the clinical and histopathological features of 2 cases of Gamna-Gandy bodies located in the spleen. The diagnosis was made by the presence of circumscribed nodules containing dark brown rod-shaped and "bamboo-shaped" fibrotic structures. These structures were positive with Perls and von Kossa staining. Differential diagnosis includes filamentous mycoses such as mucormycosis and phaeohyphomycosis. Gamna-Gandy nodules can be isolated or these bodies can be observed associated with another lesion, particularly, a tumor.     

Solitary solid stromal gastrointestinal tumors in von Recklinghausen's disease with minimal smooth muscle differentiation

Neurofibromatosis (von Recklinghausen's disease) is occasionally associated with large, solid stromal tumors of the gastrointestinal tract. The authors examined by electron microscopy two such cases of cellular spindle cell neoplasms of the small bowel histologically that resembled leiomyomas, in an attempt to clarify the cell of origin of these lesions. Ultrastructurally, the tumor cells predominantly contained moderate to large numbers of intracellular filaments, small cell processes, discontinuous adherent dense basement-membrane-like material, and abundant intercellular collagen. Definite fusiform dense bodies or structures highly suggestive of them and pinocytotic vesicles were seen in rare cells of each lesion after viewing multiple blocks. While patients with neurofibromatosis are certainly at risk of developing gastrointestinal Schwann cell neoplasms, these two cases suggest that they are also at risk for developing poorly differentiated stromal tumors, resembling leiomyomas by light microscopy, which may show only characteristic cytoplasmic differentiation of smooth muscle cells after ultrastructural examination of many sections.     

Colorectal polyps with extensive absorptive enterocyte differentiation: histologically distinct variant of hyperplastic polyps

BACKGROUND: The histologic classification of colorectal polyps is well established. However, practicing pathologists may still occasionally encounter colorectal polyps that are difficult to classify. We studied 6 colorectal polyps that showed uncommon histologic features that have not been described in the English language literature. MATERIALS AND METHODS: The polyps were studied using standard hematoxylin-eosin stain, mucin histochemistry, and electron microscopy. RESULTS: The 6 polyps we studied showed extensive papillary and villous structures with alternating villi and crypts. The villi were lined by well-differentiated absorptive cells, whereas the crypts were lined by immature glandular cells, thus mimicking the histology of the small intestinal mucosa. CONCLUSIONS: These polyps appear to represent a variant of the hyperplastic polyp, in as much as cellular maturation (immature glandular cells differentiate into the mature surface absorptive cells) is the essential feature distinguishing hyperplastic polyps from adenomas.     

Poorly differentiated squamous cell carcinoma of the nipple: a unique case for marked exophytic growth, but little invasion with neuroendocrine differentiation

A 73-year-old woman showed marked exophytic growth of a tumor (25 × 23 × 14 mm) of the nipple over a period of 2 months. Histologically, numerous tumor nodules with no apparent keratinization were observed in the exophytic lesion. The tumor cells also showed little invasion to the dermis and no metastasis to the axillary lymph nodes (LN). The tumor cells were immunohistochemically positive for cytokeratins (CKs; AE1/AE3 and 34βE12), epithelial membrane antigen (EMA), and p53, but negative for Ber-EP4 and human papilloma virus (HPV). The MIB-1 index was 56%. Some tumor cells were also positive for some neuroendocrine markers, and showed some tonofilaments and neurosecretory granules in the cytoplasm under electron microscopy. We made the differential diagnosis of mammary ductal carcinoma, basal cell carcinoma (BCC), Paget’s disease, and neuroendocrine carcinoma including Merkel cell carcinoma. The final diagnosis was poorly differentiated squamous cell carcinoma (SCC) showing exophytic growth with neuroendocrine differentiation (ND) in the nipple. To our knowledge, although only five cases of Bowen’s disease have been reported in the nipple, such a unique SCC has not been reported previously.     

Evidence for apoptosis in human atherogenesis and in a rat vascular injury model.

Apoptosis is a physiological cell death process important for normal development and involved in many pathological conditions. In atherosclerosis, pathological accumulation of cells in the intima has been attributed to the migration and proliferation of smooth muscle cells, macrophages, and lymphocytes. In this report, we explored the possibility that apoptosis may also contribute to the pathogenesis of this disease. We examined 35 human atherosclerotic lesion samples and identified a substantial number of cells undergoing apoptosis in 25 of the samples. Furthermore, in a rat vascular injury model, apoptotic cells were specifically identified in the neointima. The presence of apoptotic cells was demonstrated by terminal deoxynucleotidyl transferase-mediated dUTP-biotin nick end labeling, nuclear staining with propidium iodide, and electron microscopy. Immunostaining with cell-type-specific markers and subsequent terminal deoxynucleotidyl transferase-mediated dUTP-biotin nick end labeling analysis on the same sample revealed that the majority of the apoptotic cells were modulated smooth muscle cells as well as macrophages. These results indicate that apoptosis occurs in cells of the injured blood vessel as well as the advanced atherosclerotic lesion and that physiological cell death may have an important role in determining the course of atherogenesis.     

Fibroblasts and antigen-presenting cells in the renal interstitium of streptozotocin-induced diabetic rats on a high cholesterol diet

The cortical peritubular interstitium of the normal kidney contains both fibroblasts and antigen-presenting dendritic cells. Characteristics of these interstitial cells were analyzed in an overnutrition model by electron microscopy after the cold-dehydration technique and immunohistochemistry for antigen-presenting cells. In control rats, fibroblasts and dendritic cells were clearly identified by electron microscopy on the basis of their distinct ultrastructures. Fibroblasts possessed slender cell processes, and contained an abundance of actin filament bundles occasionally anchoring to surrounding structures, whereas dendritic cells possessed irregularly-shaped cell processes with a clear cytoplasm and a paucity of actin filament bundles. In the experimental kidney from diabetic rats given a high cholesterol diet, the peritubular interstitium contained fibroblasts and vacuolated cells, and the extracellular matrices such as collagen bundles were distinctly increased compared with the control rat kidney. Immunohistochemical staining with OX6 and ED1 revealed that the peritubular interstitium in the control rat kidney contained dendritic cells, while that in the experimental rats was occupied by macrophages. The present study provides the first evidence indicating that overnutrition may dramatically affect the immune cells in nonlymphoid tissue.     

Indeterminate cell tumor of the spleen

Indeterminate cell tumor is an extremely rare neoplasm that mainly occurs in the skin. We report a case of indeterminate cell tumor arising from the spleen, a previously unreported site for indeterminate cell tumor. Histologically, the tumor showed nests, nodules, and sheets of large polygonal cells with mostly oval nuclei; open chromatin; variable nucleoli; and abundant, eosinophilic cytoplasm. Some cells possessed irregularly convoluted nuclei with nuclear grooves and granular cytoplasm, suggestive of Langerhans cells. Immunohistochemically, the tumor cells were diffusely positive for S-100 and CD1a and negative for Langerin. No Birbeck granules were found by electron microscopy. Clinical and radiologic examination showed no other organomegaly or lymphadenopathy. A diagnosis of primary indeterminate cell tumor of the spleen was rendered. To the best of our knowledge, this is the first indeterminate cell tumor reported in the spleen. Biologic insights into dendritic cells in the spleen and the pertinent literature on these entities are reviewed.