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Immunolocalization of cathepsin D in normal and neoplastic human tissues.

The aspartic proteinase cathepsin D was purified from human spleen and localised in various formalin fixed paraffin embedded human tissues using the peroxidase-antiperoxidase (PAP) technique. Cathepsin D was shown not only in macrophages but also in other connective tissue cells, and in epithelium. It was present in spleen (littoral cells and cells within Malpighian bodies), liver (hepatocytes and Kupffer cells), lung (alveolar macrophages and bronchial epithelium), brain (neurones), lymph nodes (histiocytes in germinal centres, sinusoid lining cells) and stomach (parietal and mucous neck cells). Cathepsin D was also found in carcinomas of bronchus, stomach, colon, kidney, breast, ovary, bladder and pancreas, both in neoplastic epithelium and in stromal cells, but was seldom present in connective tissue neoplasms. A group of malignant lymphomas also contained the enzyme within scattered cells. The distribution of cathepsin D seems to be much wider than that of the structurally related aspartic proteinases pepsin, gastricsin, and renin.

Brain↗

Primary biphasic synovial sarcoma of the orbit.

Synovial sarcoma is one of the most common soft tissue malignancies of adolescents and young adults. Despite its name, it is no longer thought to be histogenetically derived from the synovium. What seems to be the first case of synovial sarcoma to arise in the orbit presented in a 21 year old woman as a slowly enlarging subconjunctival mass. Although this tumour was typically biphasic, the monophasic spindle cell variant arising at this site could easily be confused with less aggressive orbital connective tissue neoplasms.

Adult↗

Oncogenic osteomalacia: localization of underlying peripheral mesenchymal tumors with use of Tc 99m sestamibi scintigraphy.

OBJECTIVE: To highlight a strategy for potential detection of mesenchymal tumors in oncogenic malacia, as illustrated by 3 cases. METHODS: Three case reports are presented in which successful localization of the offending neoplasm was accomplished by using whole-body Tc 99m sestamibi scanning. Alternative localization techniques are also reviewed. RESULTS: Oncogenic osteomalacia occurs infrequently and is caused by neoplasms that secrete phosphatonins, substances that interfere with proximal tubular resorption of phosphorus and can result in phosphaturia, hypophosphatemia, reduced 1,25-dihydroxyvitamin D concentration, and osteomalacia. Removal of the underlying neoplasm results in complete resolution of all biochemical, pathologic, and physical manifestations of this disorder, as shown in our 3 patients. Because the neoplasms are small and can occur in any tissue compartment, they are difficult to localize, a feature that often results in therapeutic failure. CONCLUSION: We conclude that use of whole-body Tc 99m sestamibi scanning may be an appropriate and cost-effective initial strategy for the localization of peripheral phosphatonin-secreting tumors.

Aged↗

Miscellaneous rare paratesticular tumors.

A few uncommon but distinctive tumors may preferentially involve the paratestis. The 3 unusual tumors that represent the focus of this discussion are the ovarian-type epithelial tumors (OTET), the desmoplastic small round cell tumor (DSRCT), and the melanotic neuroectodermal tumor of infancy (MNTI). The OTETs are testicular homologues of their more common namesake counterparts that arise in the ovary. Most frequent of these are serous tumors of borderline malignancy, with fewer cases of serous carcinomas or other forms of mullerian differentiation. DSRCT is an increasingly recognized, aggressive, "small blue cell" neoplasm with distinctive clinical and pathologic features. These polyphenotypic tumors characteristically, but not invariably, arise in intimate association with the serosal membrane of the peritoneal cavity and harbor a signature translocation-t(11;22)(p13,q12). In the paratestis they often involve the surface of the epididymis. The MNTI is an enigmatic, histologically distinctive, low-grade neoplasm occasionally encountered in the epididymis. Recognition of its features is essential to avoid misdiagnosis as a more aggressive "small blue cell" neoplasm and consequent therapeutic mismanagement. Primary hematopoietic tumors of the paratesticular structures are rare. There appears to be a tendency for young men to have low-grade lymphomas with an indolent course and older patients to develop higher-grade tumors. Plasmacytoma and granulocytic sarcoma of the paratestis are even more rare and are often susceptible to misinterpretation. Finally, metastatic tumors and a variety of other very rare neoplasms are discussed.

Adolescent↗

Apoptosis, bcl-2 expression, and p53 expression in gastrointestinal stromal/smooth muscle tumors.

The authors investigated the relations between outcome and apoptosis, immunohistochemical demonstration of bcl-2 protein, and immunohistochemical staining for p53 protein in patients with gastrointestinal stromal/smooth muscle tumors (GIST). Patients whose tumors demonstrated cellular apoptosis using the terminal deoxynucleotidyl transferase-mediated 2'-deoxyuridine 5'-triphosphate-biotin nick end labeling (TUNEL) assay had an improved survival over those whose tumors did not improve. In contrast, patients whose tumors demonstrated staining for bcl-2 protein had a decreased survival compared with those whose tumors did not demonstrate bcl-2. There was no relation between p53 immunoreactivity and survival. These results suggest that inhibition of apoptosis may be associated with malignant behavior in patients with gastrointestinal stromal/smooth muscle tumors.

Apoptosis↗

Histopathology of laryngeal tumours.

A description is given of the frequent as well as of the less frequent epithelial and mesenchymal tumours of the larynx. The description is based on the light microscopical and histochemical characteristics of these tumours. The relation to the clinical presentation is briefly mentioned. The correlation between the subtypes and prognosis is discussed.

Carcinoma↗

Pediatric orbital tumors.

The proper management of orbital tumors in children requires a knowledge of the lesions commonly seen in this area, in addition to the appropriate methods of investigation and treatment. A multidisciplinary approach is most beneficial in obtaining the desired results.

Child↗

Myxomas in the fingertips.

The myxoma, a benign connective-tissue neoplasm consisting of stellate cells and a loose mucoid stroma, rarely involves the hand. Only three previous cases of myxoma in the finger have been reported in the literature. Two cases of myxoma in the fingers are presented here, one occurring in the finger pulp and the other in the subungual region. Cases described in the literature and the authors' experience indicate that the tumor is cured by a meticulous, marginal resection, though slight nail deformity can be a sequela.

Adult↗

Histopathology and ultrastructure of tumours and tumour-like lesions of bone.

The monograph summarizes the most important data and experience based on the clinicopathological analysis, histological and histoenzymatic examinations of more than 1000 primary tumours and 400 tumour-like lesions of bones. The diagnosis of them has been based on the WHO classification of bone tumours which appeared to be the best in meeting the needs of diagnostic practice. However, in our collection of cases also occurred such cases which did not fit into the WHO diagnostic scheme. They included lesions the separate existence of which could be proved only recently or has remained the matter of discussion, such as periosteal osteosarcoma, clear-cell and dedifferentiated chondrosarcoma, malignant fibrous histiocytoma, the so-called parachordoma, resorptive giant-cell granuloma, reactive periostitis of tubular bones of hands and feet, and the tibial lesion reminiscent of cementifying fibroma. In the majority of types of the tumours and tumour-like lesions of bones electron microscopical examination was carried out (203 cases), the results of which are presented here in a considerable extent. On the one hand, electron microscopy facilitated the diagnosis in some cases, and, on the other hand, it proved as extremely important in solving the problems of histogenesis of some of the lesions.

Bone Marrow Diseases↗

[Differential diagnosis of facial skin swellings (author's transl)].

Enlargement of the cheeks may be due to a multitude of disorders, congenital, neoplastic, and in particular inflammatory. Congenital facial anomalies include cutaneous (and osseous) hemihypertrophy of the face and unilateral angiomatous malformations (e.g. Sturge-Weber-Krabbe Syndrome). Buccal enlargement due to dermal tumours include localized haemangiomas and lymphangiomas, lipomas and other benign connective tissue neoplasms, generalized disorders of the lymphatic or reticuloendothelial system including mycosis fungoides, reticulum cell sarcoma and other soft tissue malignancies, and cutaneous manifestations of malignant haemoblastoses, in particular chronic lymphatic leukaemia. Within the very large group of inflammatory skin swellings of the face a review is made of some bacterial pyodermias, severe forms of acne vulgaris, herpes zoster, lupus vulgaris, erysipelas, rosacea, steroid dermatitis, lupus erythematosus (discoid and systemic), toxic dermatitis, allergic eczema, urticaria, Quincke's oedema, and the Melkersson-Rosenthal syndrome. The importance of prevention and early detection of steroid-induced dermatitis is emphasized. This disorder, which is a pseudo-inflammatory disfiguring complication of prolonged topical steroid abuse, ranks in frequency with the skin problems most often seen in dermatological practice.

Adult↗