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Biomedical subjects

T Mentzel

Publications and source records attributed to T Mentzel.

At least 19 recordsLinked to original sources
PubMed2005

Palmar atypical lipomatous tumour with spindle cell features (well-differentiated spindle cell liposarcoma): a rare neoplasm arising in an unusual anatomical location.

Lipomatous tumours, both benign and malignant, arising on the hands are uncommon. We present a rare atypical lipomatous tumour with spindle cell features (synonym: well-differentiated spindle cell liposarcoma) arising on the left palm of a 54-year-old male patient. The neoplasm presented as a long-standing, exophytic neoplasm measuring 9 x 9 cm. The well-circumscribed neoplasm was completely excised, and margins were tumour free. Histologically, the neoplasm showed features closely resembling spindle cell lipoma, being composed of mature adipocytic cells associated with bland, neuroid spindle cells staining positively for CD34. However, focally, atypia of adipocytic and stromal cells as well as scattered lipoblasts were noted, and immunohistochemical stainings showed focal overexpression of MDM 2 and CDK4. Aypical lipomatous tumour with spindle cell features may arise very rarely in palmar location and has to be distinguished from a number of benign and malignant mesenchymal neoplasms.

Diagnosis, Differential↗
PubMed2005

[Perivascular neoplasms of skin and soft tissues. A review].

Perivascular neoplasms of skin and soft tissues comprise a group of benign and malignant mesenchymal neoplasms showing a perivascular myogenic differentiation. Whereas glomus tumour including its variants represents a clearly defined clinicopathological entity, the whole concept of haemangiopericytoma has been questioned in the last years, and no clear diagnostic criteria are established at the moment. Myopericytomas and myofibromas represent a morphological spectrum of perivascular spindle-shaped lesions. Infantile myofibroma/infantile myofibromatosis and solitary myofibroma of adults are characterized by a biphasic growth of mature, spindled, myofibroblastic cells and smaller, immature, mesenchymal cells associated with numerous haemangiopericytoma-like vessels. Benign and rare malignant myopericytomas of skin and soft tissues are characterized by a concentric, perivascular growth of plump spindled and round tumour cells staining positively for muscle actin, alpha-smooth muscle actin and more rarely for desmin. PEComas represent an enigmatic family of neoplasms composed of perivascular epithelioid, clear, and spindled tumour cells characterized by a coexpression of melanocytic and myogenic markers.

Diagnosis, Differential↗
PubMed2005

[Myoepithelial neoplasms of skin and soft tissues].

Myoepithelial neoplasms of skin and soft tissues comprise cutaneous mixed tumor (chondroid syringoma), mixed tumor of subcutaneous and deep soft tissues, myoepithelioma and rare malignant myoepithelioma (myoepithelial carcinoma). Myoepithelial tumors of skin and soft tissues are characterized by an extreme clinicopathological heterogeneity as in other anatomic locations. The neoplasms arise in childhood as well as in adults and are composed of epithelioid, histiocytoid, spindled, plasmocytoid and/or clear tumour cells in varying combinations, and are set in a myxoid or hyalinised intercellular matrix. Immunohistochemically, neoplastic cells stain positively for epithelial markers (pancytokeratin and/or epithelial membrane antigen), and often for S 100 protein. More rarely muscle actin, glial fibrillary acid protein, calponin, and p63 are expressed, whereas desmin is usually negative. The presence of at least moderate cytological atypia is associated with a significant risk for aggressive behavior and propensity for metastasis.

Adult↗
PubMed2005

Cutaneous clear cell myomelanocytic tumour: a new member of the growing family of perivascular epithelioid cell tumours (PEComas). Clinicopathological and immunohistochemical analysis of seven cases.

AIMS: To analyse seven cases of cutaneous myomelanocytic tumour histologically and immunohistochemically. Perivascular epithelioid cell tumours (so-called PEComas) are rare and recently delineated neoplasms occurring in the lung, kidney, pancreas, uterus, falciform ligament, vulva, heart, prostate and soft tissues. PEComas are characterized by a perivascular location of neoplastic cells showing a broad spectrum of epithelioid and spindled cells with clear, and granular pale eosinophilic cytoplasm, and a variable expression of melanocytic and muscle markers, whereas S100 protein and cytokeratins are usually absent. METHODS AND RESULTS: We report seven cases of cutaneous myomelanocytic tumour arising on the lower (six cases) and upper (one case) extremities of female adults (age range 30-66 years). In all cases an ill-defined dermal lesion with extension into subcutaneous tissue was noted. The neoplasms contained numerous blood vessels with a lace-like pattern and slightly thickened vessel walls, and were composed of perivascular epithelioid cells containing clear or focally granular pale eosinophilic cytoplasm and round vesicular nuclei with small, sometimes slightly enlarged nucleoli. Increased proliferative activity and tumour necrosis were not seen. Immunohistochemically, tumour cells stained positively for HMB-45, microphthalmia transcription factor, and NKIC3 in all cases, whereas perivascular expression of alpha-smooth muscle actin and focal positivity for desmin were noted in one case each only. Two out of four cases tested stained focally positive for calponin. No expression of S100 protein and pancytokeratin was present. Despite incomplete/marginal excision in three cases none of the neoplasms has recurred locally so far. CONCLUSIONS: With the presented series of cutaneous myomelanocytic tumours the clinicopathological spectrum of PEComas is expanded.

Adenocarcinoma, Clear Cell↗
PubMed2004

[Skin adnexal and salivary gland neoplasms. Similarities and differences of selected patients].

Benign and malignant skin adnexal neoplasms, especially glandular lesions, show morphologically striking similarities to salivary gland tumors. On the other hand, histological and clinical differences are evident, and knowledge of their existence is important for adequate treatment and reliable prognostication. In this review similarities and differences of selected entities are briefly described and discussed. The following entities are reviewed: cylindroma (vs. membranous variant of basal cell adenoma), sebaceoma (vs. sebaceous adenoma), syringocystadenoma papilliferum (vs. sialadenoma papilliferum), chondroid syringoma (vs. pleomorphic adenoma), cutaneous myoepithelioma (vs. myoepithelioma of salivary glands), cutaneous malignant myoepithelioma (vs. malignant myoepithelioma of salivary glands), cutaneous adenoid cystic carcinoma (vs. adenoid cystic carcinoma of salivary glands), and mucinous eccrine carcinoma (vs. mucous carcinoma of salivary glands).

Adenoma↗
PubMed2003

Expression of drug resistance related proteins in sarcomas of the pulmonary artery and poorly differentiated leiomyosarcomas of other origin.

Sarcomas are known to develop resistance to current chemotherapeutic strategies, displaying a multidrug-resistant phenotype. Mechanisms involved in drug resistance include reduced cellular drug accumulation, drug detoxification as well as alterations in drug target specificity. In seven sarcomas of the pulmonary artery (SPA) and ten leiomyosarcomas of other origin, we studied the immunohistochemical expression of P-glycoprotein (P-gp), multidrug-resistance protein (MRP), lung resistance protein (LRP), metallothionein (MT) and topoisomerase IIalpha. Upregulation was found in tumour cells for P-gp but not for MRP in SPA and other leiomyosarcomas. Topoisomerase IIalpha was expressed at high levels in tissue of primary tumours as well as recurrent tumours. Both P-gp and topoisomerase IIalpha were present in numerous tumour-associated vessels. LRP was expressed at high levels in SPA but to a lesser extent in the other leiomyosarcomas. MT was expressed at low levels but was markedly present at the border of necrosis. The overall survival and the relapse-free survival did not correlate with the expression of these factors. There was no significant relationship between treated and non-treated patients with respect to the expression of the examined molecules. P-gp, but not MRP, may play a role in the development of drug resistance. P-gp, LRP and topoisomerase IIalpha contribute to drug resistance through expression in tumour-associated vessels. Unique high levels of topisomerase IIalpha reflect the high proliferation rate of these tumours. MT seems to serve as a detoxifying agent of metabolites at the border of necrosis. Our findings underline the fact that multiple factors contribute to chemoresistance and that examination of a spectrum of relevant molecules is probably necessary to plan the best therapy.

ATP Binding Cassette Transporter, Subfamily B↗
PubMed2003

[Cutaneous perineurioma. Clinical and histological findings and differential diagnosis].

Perineuriomas, composed almost exclusively of EMA (epithelial membrane antigen) positive cells, represent a rare and distinct entity in the spectrum of nerve sheath tumours. At present three subtypes, including intraneural and extraneural perineurioma as well as sclerosing perineurioma, can be distinguished; atypical and malignant perineuriomas are extremely uncommon. We analysed the clinicopathological and immunohistochemcial features of 13 cases of cutaneous perineurioma. The neoplasms arose in adult patients (age range from 18 to 71 years) and were seen on the hand (six cases), the thigh (three cases), the lower leg (two cases), the forearm (one case) and in perinasal location (one case). Morphologically, seven neoplasms showed features of dermatofibroma-like perineurioma, four lesions were consistent with sclerosing perineurioma, one cellular lesion resembled a solitary fibrous tumour, and one case was diagnosed as atypical/malignant cutaneous perineurioma; no local recurrences or tumour progression has been reported so far. The differential diagnosis of various variants of cutaneous perineurioma from other mesenchymal lesions as well as melanocytic and epithelial neoplasms is discussed.

Adolescent↗
PubMed2003

[Myofibroblastic sarcoma of the thoracic wall. Change in appearance in tumour recurrence].

The existence of malignant mesenchymal tumours with myofibroblastic differentiation (myofibroblastic sarcoma, myofibrosarcoma) is controversially discussed. In the present case report a low-grade myofibroblastic sarcoma of the thoracic wall with varying morphological appearance in numerous recurrences over a number of years is described. The varying immunophenotypes of tumour cells with myofibroblastic differentiation suggests that changing of morphological and immunohistochemical phenotypes may occur in tumour cells with myofibroblastic characteristics corresponding to the "plasticity" of the myofibroblast. The classification of soft tissue tumours is based on a discernible line of differentiation. If cells of a malignant mesenchymal tumour show characteristic features of myofibroblasts by light microscopy and immunohistochemistry, this neoplasm should be classified as a myofibroblastic sarcoma, even if the ultrastructural detection of "fibronexus" is not possible.

Antigens, Neoplasm↗
PubMed2003

Blastic natural killer-cell lymphoma of the skin associated with myelodysplastic syndrome or myelogenous leukaemia: a coincidence or more?

We report three patients with blastic natural killer (NK)-cell lymphoma of the skin associated with myelodysplastic syndrome (MDS) (two patients) or subacute myelomonocytic leukaemia (one patient). In two patients MDS was diagnosed before skin lesions; the patient with leukaemia initially presented with skin lesions. Our patients had several clinical features in common, namely multiple skin lesions with a bruise-like appearance, involvement of the oral mucosa, and good general status at presentation but very rapid deterioration in the course of the disease. All patients died of disease 4-14 months after the diagnosis. Histopathologically, there were cutaneous infiltrates of slightly pleomorphic medium-sized cells expressing CD4, CD56, terminal deoxynucleotidyl transferase (focally) and being negative for surface CD3, cytotoxic molecules, B-cell-associated markers and myelomonocytic markers. Erythrocyte extravasation was seen in all patients. T-cell receptor genes were in germline configuration. MDS was classified as refractory cytopenia with multilineage dysplasia and ring sideroblasts and refractory anaemia with transition to refractory anaemia with excess of blasts. We reviewed similar cases reported in the literature showing coexistence of blastic NK-cell lymphoma/leukaemia and MDS or myelogenous leukaemia. We conclude that given an overall limited number of reported cases of blastic NK-cell lymphoma/leukaemia, its association with various myelodysplastic/myeloproliferative disorders seen in a subset of patients ( approximately 15-20%) is more than coincidental and may indicate their common origin.

Aged↗
PubMed2003

Haemorrhagic dermatomyofibroma (plaque-like dermal fibromatosis): clinicopathological and immunohistochemical analysis of three cases resembling plaque-stage Kaposi's sarcoma.

AIMS: Dermatomyofibroma (plaque-like dermal fibromatosis) represents a distinct clinicopathological entity in the spectrum of cutaneous mesenchymal neoplasms showing a myofibroblastic line of differentiation. These benign neoplasms occur frequently, but not exclusively, in young women, and the shoulder girdle as well as the upper trunk are common locations. Histologically, dermatomyofibroma is characterized by a plaque-like proliferation of cytologically bland spindle-shaped tumour cells containing an ill-defined, pale eosinophilic cytoplasm and elongated, neuroid nuclei. Neoplastic cells are arranged in bundles and fascicles orientated parallel to the skin surface, adnexal structures are spared and elastic fibres are increased and fragmented. Immunohistochemically, tumour cells express vimentin and variably muscle actin and alpha-smooth muscle actin, but are negative for desmin, CD34, S100, and epithelial markers. The main differential diagnosis includes hypertrophic scar, dermatofibroma (fibrous histiocytoma), pilar leiomyoma, neurofibroma, adult myofibromatosis, extra-abdominal fibromatosis and plaque-stage dermatofibrosarcoma protuberans. METHODS AND RESULTS: We report three cases of dermatofibroma arising in male patients aged 31, 36, and 47 years on the thigh, chest wall and back, respectively. All lesions were completely excised and no local recurrence has been reported. Histologically, the neoplasms showed classical features of dermatomyofibroma; however, in addition abundant extravasated erythrocytes, scattered inflammatory cells, numerous capillaries, and sieve- and slit-like spaces, features resembling plaque-stage Kaposi's sarcoma, were noted. In none of the cases did spindled tumour cells stain positively for CD34, and HHV8 was not detected by polymerase chain reaction. CONCLUSIONS: The reported cases widen the clinicopathological spectrum of dermatomyofibroma and emphasize plaque-stage Kaposi's sarcoma as an additional differential diagnosis.

Adult↗
PubMed2002

[Diffuse dermal angiomatosis].

Diffuse dermal angiomatosis (DDA) is a benign, acquired vascular proliferation characterized by painful, poorly circumscribed, livid-erythematous plaques with frequent central ulceration. The clinical features are reminiscent of reactive angioendotheliomatosis or embolia cutis medicamentosa. Histologically, a dense diffuse network of regular capillary vessels throughout the dermis is seen. Endothelial atypia, atypical mitoses or signs of vasculitis are missing. A 43-year-old woman developed a bizarre brownish-livid rapidly growing lesion of DDA 8 centimeters above a surgical scar, shortly after removal of 20 kilograms of fatty tissue from the lower abdominal wall. DDA regressed spontaneously within 12 weeks, and has not recurred over 4 years of follow-up.

Abdominal Wall↗
PubMed2002

[Tumors of the lymphatic vessel of the skin and soft tissue].

Tumours of lymphatic vessels comprise only a small group in the heterogeneous spectrum of vascular neoplasms of skin and soft tissues. However, this discrepancy between haemangiomas/angiosarcomas and lymphangiomas/lymphangiosarcomas probably represents the present inability to reliably differentiate between lymphatic and capillary vascular endothelium. Histologically, neoplastic lymphatic vessels tend to be lined by endothelial cells with plumper and more prominent, matchstick-like nuclei (in contrast to vascular spaces in haemangiomas that are lined by flat or epithelioid endothelial cells), often show variations in the thickness of the vessel walls and are not completely surrounded by actin-positive (myo)pericytes. Endothelial cells in lymphatic neoplasms tend to be negative for CD34 or stain only focally positive for this marker and an expression of lymphatic markers as vascular endothelial growth factor-C receptor (VEGRF-3), podoplanin and M2A oncofetal antigen has been reported most recently. In addition to "traditional" lymphatic neoplasms including lymphangioma circumscriptum, cavernous lymphangioma/cystic hygroma, benign lymphangioendothelioma, lymphangiomatosis and the rare lymphangiosarcoma, histological and immunohistochemical features of a group of vascular neoplasms with a hobnail cytomorphology (hobnail haemangioma, retiform haemangioendothelioma, papillary intralymphatic angioendothelioma, benign lymphangiomatous papule following radiotherapy) suggest that these lesions also belong to the spectrum of tumours of lymphatic vessels.

Diagnosis, Differential↗
PubMed2002

[Cutaneous mesenchymal neoplasms versus mesenchymal neoplasms of subcutaneous and deep soft tissue. Similarities and differences].

As in subcutaneous and deep soft tissues, cutaneous mesenchymal neoplasms are also relatively rare and characterized by a clinicopathological heterogeneity. There are striking differences in regard to the occurrence of different lines of differentiation of mesenchymal neoplasms in superficial and deep locations, and some distinct entities arise exclusively in the dermis. Superficially located mesenchymal neoplasms are characterized by a number of specific morphological features in comparison to deeper located lesions. Clinically important, dermal sarcomas of some lines of differentiation are associated with a favorable clinical prognosis, independent of the histological grading, which is important to know for the correct treatment of patients. In the following review similarities and distinguishing clinicopathological features of superficially and deeply located mesenchymal tumors are discussed.

Diagnosis, Differential↗
PubMed2002

Superficial acral fibromyxoma: report of two cases.

Superficial acral fibromyxoma (SAFM) is a rare soft tissue tumor that has recently been delineated as a separate entity. We report 2 cases of SAFM and discuss its pathological features and differential diagnosis. Both patients had lesions on the toe. In 1 patient, the tumor was found after nail extraction, which had been performed for the treatment of onychomycosis, whereas in the other patient the tumor itself was the reason for seeking dermatological assistance. Biopsies from both cases demonstrated similar features. There was a moderately circumscribed, non-encapsulated tumor extending through the whole dermis. The neoplasm was composed of spindle and stellate cells with slight nuclear atypia arranged in a loose storiform, partly fascicular growth pattern. In 1 case, strands of cells with rather wavy nuclei were seen at the periphery of the tumor. Mitotic figures were scarce. The neoplastic cells were embedded in a myxoid stroma with increased numbers of small blood vessels and scattered mast cells. Immunohistochemically, the tumor cells showed weak focal positivity for CD34 and stained negatively for S-100 protein and alpha-smooth muscle actin. In 1 case epithelial membrane antigen (EMA) was negative, whereas in the second case focal expression of EMA by neoplastic cells was seen. Alcian blue staining revealed abundant mucinous material within the stroma. In conclusion, SAFM represents a distinct entity in the spectrum of cutaneous myxoid tumors. The differential diagnosis of SAFM includes various myxoid neoplasms and tumors with a predilection for distal parts of the extremities.

Aged↗
PubMed2001

[Degenerative changes in parapharyngeal schwannoma "ancient schwannoma". Case report and review of the literature].

Schwannomas are rare, slow-growing, benign neoplasms derived from the schwann cells of the nerve sheath of peripheral nerves. Extracranial schwannomas are most frequently localized within the extremities or the head and neck region. For example, they are common in the skull base, the carotid sheath, and the parapharyngeal space. Beneath several histological variants, schwannomas showing prominent degenerative changes (so-called,,ancient schwannomas") are exceedingly rare. Histopathological features, such as degenerative changes and nuclear atypias in ancient schwannomas, may be easily confused with malignant mesenchymal neoplasm.We present the case of a 32-year-old female who required surgery for a parapharyngeal neoplasm. Histological examination revealed features of an ancient schwannoma.

Adult↗
PubMed2001

The association between tumour progression and vascularity in myxofibrosarcoma and myxoid/round cell liposarcoma.

Angiogenesis is an important factor in the morphological progression and metastasis of many solid tumours. We studied two homogeneous series of myxofibrosarcoma (MFS) and myxoid/round liposarcoma (MRLS), characterised by distinct vascular patterns and correlated the intratumoral microvessel density (IMD) with morphologic progression in both types of sarcoma. In our study, 43 cases of MFS and 42 cases of MRLS were graded according to established diagnostic criteria. For evaluation of IMD, representative sections were stained immunohistochemically for CD31. After selection of "neovascular hot spots", IMD was calculated by measuring the endothelial surface within twenty 200x fields in relation to the total analysed area. In addition to the correlation of IMD with histological grades of malignancy, a correlation of IMD with the inflammatory infiltrate in MFS was done. To determine whether vascular endothelial growth factor (VEGF) and its receptors, KDR and flt-1, may play a role in the progression of both types of sarcomas, we used mRNA in situ hybridisation (ISH) to study VEGF, KDR and flt-1 expression in selected cases. In addition, the expression of thrombospondin-1, which has been reported to inhibit angiogenesis, and of collagen type I was studied using mRNA ISH. Cases of MFS varied histologically from hypocellular, mainly myxoid, neoplasms (low-grade malignant, 18 cases) to intermediate-grade malignant lesions with increased cellularity and mitotic activity (13 cases), and high-grade malignant cases with marked pleomorphism, high proliferative activity and areas of necrosis in many cases (12 cases). Cases of purely low-grade myxoid liposarcoma (16 cases) were characterised by low-cellularity, mucin pooling and plexiform vasculature. In combined MRLS, these hypocellular areas were admixed with hypercellular, round cell areas (5-80% of the analysed tumour area; 23 cases), and in round cell liposarcoma (three cases) rounded tumour cells predominated (>80% of the analysed tumour area). The average IMD in intermediate and high-grade malignant MFS (4.03 and 4.09, respectively) was significantly higher than in low-grade malignant MFS (2.73). Correlation of vascularity with the inflammatory infiltrate in MFS showed increased IMD only in cases with abundant neutrophils; most of these cases were high-grade malignant neoplasms. In contrast, no statistical correlation between morphological progression and IMD was seen in myxoid liposarcoma (6.08), MRLS (6.57) and round cell liposarcoma (4.07). VEGF mRNA was expressed by tumour cells in all histological grades of MFS and MRLS. VEGF receptor mRNA was weakly expressed by endothelia of newly formed blood vessels in both entities. Interestingly, tumour cells of all analysed cases of MFS strongly expressed collagen type I and thrombospondin-1, while these proteins were not detected in tumour cells of MRLS. In conclusion, morphologic tumour progression in MFS is associated with increased IMD, whereas, in MRLS, no such correlation is seen. Whereas VEGF and VEGF receptor mRNA were expressed in both entities, a characteristic expression profile of collagen type I and thrombospondin-1 in MFS emerged. Further studies are necessary to correlate vascularity and clinical course in MFS and MRLS.

Adolescent↗
PubMed2001

Osteopontin expression in primary sarcomas of the pulmonary artery.

Primary tumors of the great vessels (aorta, pulmonal artery, and inferior vena cava) are rare and represent in most cases vascular leiomyosarcomas. Furthermore, there also exists a group of sarcomas arising from the intima, known as intimal sarcomas, associated with early metastasis and a very poor prognosis. Osteopontin (OPN) is an extracellular matrix protein that binds to alphav integrins, thereby promoting cell attachment, chemotaxis, and signal transduction. The reported association of OPN with malignancy and metastasis prompted us to examine the expression of this protein in seven sarcomas of the pulmonary artery. Strong OPN-specific staining could be detected in tumor cells and the adjacent extracellular matrix. Using a double labeling procedure, proliferating cells showed a strong positive reaction with antibodies against OPN. In addition, this protein could be demonstrated in the cytoplasm of macrophages. CD44, a putative receptor of OPN, was expressed on the cellular surface of tumor-associated lymphocytes. The expression of OPN in macrophages and tumor cells indicates that this molecule could possibly mediate cellular adhesion of both cell types in pulmonary sarcomas. The detection in the extracellular matrix shows that OPN is actively secreted and may interact with the corresponding receptor, CD44, on the surface of lymphocytes. Although the function of OPN is not yet fully understood, our data indicate that strong expression of this molecule in poorly differentiated sarcomas could play a role in the progression of malignancy and metastasis as described previously for carcinomas.

Adult↗