Biomedical subjects

T Mentzel

Publications and source records attributed to T Mentzel.

At least 37 records · Page 2Linked to original sources

PubMed2001

Vascular tumors simulating occlusive disease.

Two cases of vascular tumors of large vessels with intraluminal growth simulating venous thrombosis and arterial occlusive disease are reported. One was a borderline malignant epithelioid hemangioendothelioma of the femoral vein and the other a malignant epithelioid angiosarcoma of the carotid artery. Immunohistochemical studies permitted to classify the tumors. Treatment consisted in surgical resection. No recurrence and no metastasis are noted at 24 months. Uncertainty regarding biological behaviour of vascular tumors and treatment persists.

Adult↗

PubMed2001

Pleomorphic rhabdomyosarcoma in adults: a clinicopathologic study of 38 cases with emphasis on morphologic variants and recent skeletal muscle-specific markers.

Pleomorphic rhabdomyosarcoma (PRMS) is a rare and controversial tumor of skeletal muscle phenotype. Diagnostic criteria for PRMS by combined histology and currently available immunohistochemistry have not been clearly defined. We report 38 pleomorphic rhabdomyosarcomas in adults, explore morphologic variants, and discuss our experience with both specific and nonspecific skeletal muscle markers in these tumors. Clinical data, morphology, and immunohistochemistry were reviewed. Electron microscopy was performed. Of 38 cases, there were 28 males and 10 females. Patient ages ranged from 21 to 81 years (median = 54 y; mean = 51 y). Tumors were located in the lower extremity (n = 18), abdomen/retroperitoneum (n = 6), chest/abdominal wall (n = 5), spermatic cord/testes (n = 4), upper extremity (n = 3), and one each in the mouth and orbit. Tumor sizes ranged from 1.5 to 15.0 cm (mean = 7.3 cm; median = 6.8 cm). The cases were divided into three variants, each with large, atypical, pleomorphic polygonal rhabdomyoblasts (PRMB) with abundant eosinophilic cytoplasm in varying numbers and different morphologic backgrounds of round or spindled rhabdomyoblasts (RMB). 1. Classic PRMS: Predominantly atypical PRMB in sheets (n = 8). 2. Round cell PRMS: Clusters of PRMB throughout the tumor with a background of slightly atypical, medium-sized, round, blue RMB (n = 13). 3. Spindle cell PRMS: Scattered PRMB in a predominance of atypical spindled RMB arranged in a storiform growth pattern (n = 17). Immunohistochemistry revealed the following: myoglobin (37/38), MyoD1 (19/36), skeletal muscle myogenin (myf4; 19/34), fast skeletal muscle myosin (4/5), desmin (36/38), muscle-specific actin (MSA; 25/35), smooth muscle actin (SMA; 15/33), and muscle specific myogenin (myf3; 25/35). Immunohistochemistry was supportive of skeletal muscle differentiation with at least one positive skeletal muscle-specific marker (myoglobin, MyoD1, fast skeletal muscle myosin, or myf4). In addition, all cases had some positivity for nonspecific muscle markers (desmin, MSA, SMA, myf3). Electron microscopy (EM), performed on eight selected cases from all three morphologic groups, demonstrated definitive skeletal muscle differentiation in all cases. Follow-up, available on 30 (79%) cases, revealed that 70% of patients died of disease (mean 20 months, range 1 month-108 months), 3% were alive with disease at 12 months (n = 1); and 27% had no evidence of disease (mean, 83 mo; range, 18 to 108 mo). PRMS, a tumor of predominantly middle-aged adult males in the lower extremity, can be diagnosed by the morphologic presence of scattered PRMB with immunohistochemical evidence of at least one skeletal muscle-specific marker. There are three morphologic variants of PRMS. The appropriate diagnosis of PRMS is significant as it is a high-grade sarcoma, with an aggressive clinical course.

Actins↗

PubMed2001

Benign fibrous histiocytoma (dermatofibroma) of the face: clinicopathologic and immunohistochemical study of 34 cases associated with an aggressive clinical course.

Thirty-four cases of fibrous histiocytoma (dermatofibroma) arising on the face are reported. These neoplasms occurred frequently in females (24 female, 10 male) and showed a broad age range (12 to 85 years; mean: 43.6 years, median: 41 years). The neoplasms originated on the forehead (nine cases), the cheek (eight cases), the eyebrow (four cases), the temporal region (three cases), the nose (two cases), and the ear (one case); in seven cases the location face was given only. Five of 27 cases with follow-up information (median: 5 years) recurred locally; in one case four recurrences were excised within 8 years. The majority of cases extended into the subcutis and deep soft tissue including striated muscle (50% of cases). Histologically, only the minority of cases was composed entirely of histiocytoid and spindle-shaped tumor cells arranged in a storiform growth pattern. In many cases cellular fascicles and bundles of spindle-shaped tumor cells were noted in addition to classical morphological features of fibrous histiocytoma. A moderate mitotic rate (mean: 2.97 mitoses in 10 HPFs) was observed, and in few cases increased atypia was evident. Frank tumor necrosis and/or vascular invasion were not identified. Immunohistochemical studies revealed Factor XIIIa positivity in 13 out of 17, focal CD68 positivity in 6 out of 10, and alpha-smooth muscle actin positivity in 16 out of 19 cases tested. These lesions should be distinguished from dermatofibrosarcoma protuberans, including its fibrosarcomatous variant, leiomyosarcoma, and low-grade myofibroblastic sarcoma. Cases of fibrous histiocytoma of the face have to be excised with wider margins in comparison with examples of classical fibrous histiocytoma occurring on the extremities because of diffuse infiltration, involvement of deeper structures, and an increased rate of local recurrences.

Actins↗

PubMed2001

Cutaneous myoepithelioma: an under-recognized cutaneous neoplasm composed of myoepithelial cells.

Benign and malignant neoplasms of myoepithelial cells comprise a rare but well-characterized group of tumors, among which myoepithelioma of the salivary glands is the best known. Extrasalivary examples of myoepithelioma also have been described in the breast, larynx, and retroperitoneum. Recently, myoepithelioma of the soft tissue also has been reported. According to this description, myoepithelioma and mixed tumors arising in the skin and subcutis represent points along a clinicopathologic spectrum of cutaneous and soft-tissue tumors. To the best of our knowledge, there has been only one case report of an entirely cutaneous myoepithelioma in the literature. We report herein five additional examples of purely myoepithelial tumors located exclusively in the dermis. Histopathologically, the neoplasms were well-circumscribed dermal lesions composed of fascicles of spindle cells with eosinophilic cytoplasm and ovoid-to spindle-shaped nuclei. Focally, neoplastic aggregations of more epithelioid cells representing large round cells with abundant pale cytoplasm arranged in solid clusters, cords, or strands were also seen. Ductal differentiation was not identified in either of these solid aggregations of epithelioid cells or in the fascicles of spindle-shaped cells. Nuclear pleomorphism in epithelioid and spindle-cell areas was mild, and mitotic figures were very sparse. In some cases, small, necrotic areas were seen within the solid aggregations of spindle-shaped cells. Neoplastic stroma was scant and composed of fibrillary collagen and abundant mucin. In one case, the stroma consisted of clusters of mature adipocytes intermingled with fascicles of myoepithelial cells. Areas of chondroid or osteoid metaplasia were not seen in any of the cases. Immunohistochemically, neoplastic cells expressed positivity for muscle specific actin (HHF35), alpha smooth muscle actin (IA4), S-100 protein, glial fibrillary acidic protein (GFAP), and epithelial membrane antigen (EMA), whereas stains for pan-cytokeratin (MNF116) were focal and weak. The findings in this report expand the clinical and histopathologic spectrum of cutaneous myoepithelioma, an under-recognized cutaneous neoplasm of myoepithelial cells.

Adolescent↗

PubMed2001

Dendritic cell neurofibroma with pseudorosettes: a report of 18 cases of a distinct and hitherto unrecognized neurofibroma variant.

The authors present 18 cases of a hitherto unrecognized variant of cutaneous neurofibroma. The tumors presented in adults (10 occurred in men and eight occurred in women) as a solitary, well-circumscribed, superficial lesion located in the dermis measuring 3 to 17 mm (mean size, 6.2 mm). The tumors formed oval-shaped masses that ran perpendicular to the epidermis. In the deep part of the tumor there was multinodular arrangement with two types of cells: Type I cells were small, dark, lymphocyte-like cells with a slightly irregular nucleus and inconspicuous cytoplasm. Type II cells were larger, with pale-staining vesicular nuclei, with frequent invaginations and intranuclear inclusions, and had copious clear eosinophilic cytoplasm that formed a stellate growth pattern, which was poorly visible on hematoxylin and eosin staining. Type I cells were grouped concentrically around type II cells and formed pseudorosettes. Most of the type I and type II cells were S-100 protein and CD57 positive, and various proportions of both cell types were CD56 and PGP9.5 positive. All cells were chromogranin A, synaptophysin, glial fibrillary acidic protein, cytokeratins, CD1a, CD21, CD31, alpha-smooth muscle actin, muscle-specific actin, desmin, and HMB-45 negative. CD34 stained intralesional fibroblasts. Antibody to epithelial membrane antigen stained only the perineurium around the tumor masses, suggesting that the tumors arose inside the nerve sheath. No signs of neurosecretory granules were present at ultrastructural level. None of the lesions recurred and none metastasized over a mean follow-up of 8.1 years.

Adult↗

PubMed2001

Large B-cell lymphoma of the leg--complete remission with perilesional interferon alpha.

We present the case of an 85-year-old woman with cutaneous and subcutaneous nodules on the lower and upper leg showing the characteristic histological features of a large B-cell lymphoma of the leg. Investigations for non-Hodgkin's lymphoma with the prognostic markers matrix metalloproteinase 2 and soluble intercellular adhesion molecule 1 revealed an increased serum level of the latter. Monotherapy with perilesional interferon alpha(2a) 3 x 9 million units/week was well tolerated. The final outcome was a complete remission after 14 months with an overall survival of more than 17 months.

Aged↗

PubMed2001

Cutaneous lipomatous neoplasms.

Benign and malignant lipomatous tumors are the most common neoplasms of subcutaneous and deep soft tissues in adults. In contrast, purely cutaneous lipogenic neoplasms are exceptionally rare, and some entities and variants of adipocytic neoplasms have not yet been described in the skin. In addition, some dermal lipogenic neoplasms are characterized by different specific clinicopathologic features in comparison with more deeply located tumors. Cutaneous angiolipoleiomyoma ("angiomyolipoma") is seen most often in male patients and in an acral location. It is characterized histologically by mature adipocytes, thick-walled blood vessels, and smooth muscle cells arranged in well-defined fascicles and around blood vessels. Smooth muscle cells in cutaneous angiolipoleiomyoma stain negatively for HMB-45, in contrast to renal and extrarenal angiomyolipoma. Adenolipoma of the skin is a superficially located form of lipoma composed of large lobules of mature adipocytic tissue admixed with eccrine ducts and glands. Cutaneous spindle cell/pleomorphic lipomas, in contrast to subcutaneous forms, are more common in female patients and have a wide anatomic distribution. Histologically, cutaneous spindle cell/pleomorphic lipomas are characterized by an infiltrative growth pattern mimicking more aggressive lesions. Rarely, liposarcoma may occur as a purely cutaneous lesion with a favorable clinical prognosis despite high-grade morphologic features in many cases.

Adipocytes↗

PubMed2000

Biological continuum of benign, atypical, and malignant mesenchymal neoplasms - does it exist?

The characterization of specific cytogenetic and molecular abnormalities in benign and malignant soft tissue tumours has increased our understanding and knowledge of the biology of these rare neoplasms in recent years and has led to the modification of a number of traditional classification schemes. Contrary to popular belief, it is reasonable to propose that there exists a molecular, genetic, and morphological continuum of benign, atypical, and malignant mesenchymal neoplasms. The identification of characteristic molecular changes in benign lipomas, lipomas with minimal atypia, and atypical lipomatous tumours, well-differentiated liposarcomas supports the hypothesis of a stepwise process in the pathogenesis of these neoplasms. Not only are these findings important for our understanding of the biology of soft tissue tumours, but they also may increase diagnostic and prognostic accuracy.

Disease Progression↗

PubMed2000

[Symplastic hemangioma].

BACKGROUND AND OBJECTIVE: Endothelial nuclear atypia is the hallmark of malignant vascular tumors. Pleomorphic nuclei of the muscular vessel wall and the adventitia are manifestations of degenerative phenomena and should not be misinterpreted as signs of malignancy. PATIENTS/METHODS: Three long-standing solitary superficial vascular tumors (61-year-old woman, 48- and 63-year-old men) were removed by primary excision. Sections were stained according to standard histologic and immunohistologic protocols. RESULTS: Symplastic hemangiomas show the silhouette of a small superficial angioleiomyoma or capillary hemangioma. Characteristic features are multinucleate cells of the muscular vessel wall and the adventitia with pleomorphic nuclei, broad hyalinized vessel walls, and the distinctive lack of endothelial nuclear atypia. Recurrences or metastases were not reported (follow-up of 9, 45 and 90 months). CONCLUSIONS: Symplastic hemangioma is a benign superficial hemangioma with histological features of a pseudomalignancy. The distinctive lack of endothelial nuclear atypia allows distinction from malignant vascular tumors.

Angiomyoma↗

PubMed2000

[Lipomatous tumors of the skin and soft tissue. New entities and concepts].

Mesenchymal neoplasms of lipogenic differentiation are the most common soft-tissue neoplasms in adults. In recent years a number of "new" entities and variants have been described. Their recognition is important to avoid diagnostic pitfalls and inappropriate therapy. Chondroid lipoma, myolipoma, and purely cutaneous spindle-cell/pleomorphic lipoma are biologically benign neoplasms which may mimic sarcomas morphologically. Despite high-grade morphology cutaneous liposarcomas carry a favorable clinical prognosis. Spindle cell liposarcoma is a rare variant of atypical lipomatous tumour (well-differentiated liposarcoma) and must be distinguished from dedifferentiated liposarcoma with metastatic potential and benign spindle cell lipoma. The advent of cytogenetic and molecular investigations of lipomatous neoplasms has contributed to a better understanding of the biology of these neoplasms and led to a modification of conventional classification schemes.

Diagnosis, Differential↗

PubMed2000

Sclerosing, pseudovascular rhabdomyosarcoma in adults. Clinicopathological and immunohistochemical analysis of three cases.

Rhabdomyosarcoma in adults represents a rare soft tissue neoplasm which is seen most frequently in its pleomorphic subtype in this age group. Very rarely, clear cell and spindle-cell variants have been reported. In this study we describe three cases of rhabdomyosarcoma in adult patients, characterised by prominent hyaline sclerosis and a pseudovascular growth pattern. All cases were identified in the consultation files of one of the authors and routinely processed. Immunohistochemical studies were performed on paraffin sections with the alkaline phosphatase-antialkaline phosphatase method. The patients, two women and one man, were 40, 41, and 56 years old. One developed a deep-seated soft tissue mass in the left lower leg, and one, a tumour of the left upper jaw. In one patient a bone tumour in the proximal body of the sacrum without extension into soft tissues was seen. The patients were treated by wide excision, piecemeal excision and incomplete excision in one case each; additional radiotherapy was performed in all three cases, and chemotherapy in two patients. In one patient multiple pulmonary metastases were noted, which showed progression despite systemic chemotherapy. Histologically, the neoplasms were composed of round/polygonal and spindle-shaped tumour cells including typical rhabdomyoblasts. In all cases a pseudovascular pattern and prominent hyaline sclerosis of the intercellular matrix was seen. Immunohistochemically, tumour cells stained positively for desmin and muscle actin (HHF35) and also for markers of striated muscle differentiation (myogenin, MyoD1, fast myosin). In this paper an unusual morphological variant of rhabdomyosarcoma arising in adult patients is described, which should be added to the morphological spectrum of these neoplasms.

Adult↗

PubMed2000

Intradermal spindle cell/pleomorphic lipoma: a distinct subset.

Spindle cell/pleomorphic lipomas are a group of benign lipogenic tumors composed of primitive spindle cells, multinucleated giant cells, and mature adipocytes. These tumors have rarely been reported to arise in the dermis and may be misdiagnosed in this location. Twenty (12.7%) intradermal lesions identified among 157 spindle cell/pleomorphic lipomas in the authors' files were studied clinicopathologically and immunohistochemically. The patients' ages ranged from 20 to 85 years (median: 42 years); 14 of 20 patients were female (70%). Anatomical sites were the head/neck region (7 cases, 4 of which arose on the face), shoulder/upper back (4 cases), lower limbs (4 cases), trunk (3 cases), and upper limbs (2 cases). Most lesions presented as a soft and slowly enlarging cutaneous nodule, usually measuring less than 2.5 cm. Histologically, these dermal lesions differed from usual spindle cell/pleomorphic lipoma, being unencapsulated with poorly defined infiltrative margins, although the cytomorphologic findings, ropy collagen, and mast cells were as seen in usual subcutaneous lesions. Six cases showed features of pleomorphic lipoma. Immunohistochemically, lesional cells stained positively for CD34 and were negative for S-100 protein. One case recurred locally after 21 years. Dermal spindle cell/pleomorphic lipomas are distinctive in their apparent female predilection, wider anatomical distribution than subcutaneous lesions, and lack of circumscription. These unusual features can cause problems in differential diagnosis.

Adult↗

PubMed1999

[Extracranial meningioma of the parotid gland].

Extracranial meningiomas of the head and neck region are very rare, although secondary extracranial meningiomas beside meningiomas of primary extracranial origin have also been described. Trauma, radiation and (uncommonly) metastatic spread have been associated with the pathogenesis of secondary extracranial meningiomas. We present a case of a 53-year-old patient who required six neurosurgical interventions because of a recurrent intracranial meningioma. Further surgery was required for tumor in the left parotid gland. Histological examination of this neoplasm showed an atypical meningotheliomatous meningioma. The patient is now 17 months after surgery and is free of local recurrences or metastases.

Diagnosis, Differential↗

PubMed1999

[Cutaneous neural neoplasms--an update].

Until recently, benign cutaneous neural tumours which do not fulfil criteria for either neurofibrom or schwannoma often were lumped into the broad category of benign peripheral nerve sheath tumours (PNST). However, during the last years a number of new entities of neural tumours have been described, and advances in immunohistochemistry and electronmicroscopy have helped us to better understand the cytological differentiation in these neoplasms. The knowledge of these distinctive neoplasms is necessary in order to avoid diagnostic pitfalls and misdiagnosis of more aggressive neoplasms. These distinctive lesions include: neurothekeoma, which can divided into classical myxoid and cellular types showing characteristic histological and immunohistochemical features. Typical neurothekeoma (nerve sheath myxoma) is a lobular or nodular dermal neoplasm composed of plump spindled or stellated, S-100 positive tumour cells set in a maxoid stroma. In contrast, cellular neurothekeoma is characterized as an ill-defined dermal neoplasm composed of concentric nests and fascicles of spindle-shaped and epithelioid tumour cells, which are S-100 negative but stain positively for NKIC3. The evidence of intermediate forms of neurothekeoma showing features of ordinary hypocellular neurothekeoma and cellular neurothekeoma, as well as ultrastructural studies, emphasize that both variants represent a spectrum of neurothekeoma; solitary circumscribed neuroma ("palisaded encapsulated neuroma") manifests mainly as a skin-colored or pink papule or nodule, and is most often located on the face. Histologically, solitary circumscribed neuroma is a well-circumscribed round or ovoid dermal neoplasm composed of interwoven fascicles of schwann cells, which stain positively for S-100 protein and numerous neurofilament positive axons surrounded partly by fibroblasts and EMA-positive perineural cells; perineurioma is a rare well-circumscribed neoplasm which occurs mainly in subcutaneous tissue and only rarely in the dermis and in deep soft tissues. Perineurioma is composed of elongated bipolar spindle-shaped tumour cells which are arranged in storiform, whorled, linear or lamellated growth patterns, The tumour cells stain positively for vimentin and EMA, and for CD 34 in a number of cases, but lack positivity for S-100 protein, neurofilament and desmoplakin. In addition unusual forms of schwannoma (cellular schwannoma, solitary plexiform schwannoma, melanotic schwannoma) and neurofibroma ("atypical" (bizarre) neurofibroma, diffuse neurofibroma, epithelioid neurofibroma) are briefly discussed.

Humans↗

PubMed1999

[Primary synovial sarcoma of the lung].

In the biopsy material of a polypoid intrabronchial lesion of a 54-year-old male patient with radiologically established lung tumor, spindle-cell neoplasia was seen. No clear histogenetic classification of the submucous tumor process was possible. After resection of the right upper lobe of the lung, including the results of additional immunohistochemical analyses, the tumor was classified as biphasic synovial sarcoma. This was suggested mostly by immunohistochemical detection of the epithelial membrane antigene (EMA) and of vimentin. Extensive clinical examinations ruled out the possibility of a primary tumor with extrapulmonary localization. This case report shows the problems faced in correctly classifying the histogenesis or the direction of differentiation in extremely rare cases of primary pulmonary synovial sarcoma. Especially in small biopsy samples showing a submucous spindle-cell tumor of the lung, apart from frequent sarcoma metastases, the presence of primary synovial sarcoma of the lung has to be considered in the differential diagnosis.

Antigens, CD↗

PubMed1999

[Tyrosine-rich crystalloids in a myoepithelioma of the minor salivary glands in the smooth palate].

Tyrosine-rich crystalloids in tumors of the salivary glands are rare and have been reported mainly in specimens from Black African patients. The pathogenesis of these structures is still unclear, but pathological secretion by neoplastic myoepithelial cells is supposed. Millon's staining and ultrastructural examinations are used for confirming the diagnosis. We present the case report of a 59-year-old woman with a myoepithelioma of the minor salivary glands in the smooth palate containing tyrosine-rich crystalloids as an example of this rare phenomenon.

Black People↗

PubMed1999

[Chondroid lipoma. Clinicopathological, immunohistochemical, and ultrastructural analysis of six cases of a distinct entity in the spectrum of lipomas].

A series of six cases of chondroid lipoma is reported in this paper. The age of the patients, four women and two men, ranged from 34 to 75 years. Four tumours arose in the lower and two in the upper extremities between 4.5 and 8 cm in size. Whereas two neoplasms were located in the subcutis, four lesions were seen in perifascial or intramuscular tissues. Histologically, all neoplasms were encapsulated and characterized by a somewhat lobular growth pattern. The neoplasms were composed of mature adipocytes, uni - and multivacuolated lipoblasts, and nests and strands of cells reminiscent to hibernoma cells or chondrocytes. Immunohistochemically and ultrastructurally the lipogenic nature of the latter type of cells was confirmed. Interestingly, focal immunopositivity of tumour cells for cytokeratin was found in two cases. Tumour cells were set in a myxohyaline matrix showing different degrees of degeneration. Chondroid lipoma represents a distinct entity in the spectrum of lipomatous lesions and has to be distinguished from benign and malignant neoplasms (extraskeletal chondroma, hibernoma, lipoblastoma, chondrolipoangioma, myoepithelioma, myxoid/round cell liposarcoma, and extraskeletal myxoid chondrosarcoma). Because they are easily misdiagnosed as malignant tumours, familiarity with the features of chondroid lipoma is of practical importance to avoid an overtreatment.

Adipose Tissue↗

PubMed1999

Solitary fibrous tumour of the pancreas: a new member of the small group of mesenchymal pancreatic tumours.

Solitary fibrous tumours usually occur in the pleura, but occasionally they appear in extraserosal soft tissues or parenchymatous organs, where their diagnosis often causes problems. This report describes a solitary fibrous tumour (SFT) of the pancreas in a 50-year-old woman treated by left-side pancreatectomy. The tumour showed immunocytochemical reactivity for CD34, CD99 and bcl-2. Because of its favourable prognosis, SFT must be clearly distinguished from leiomyosarcoma, the most frequent nonepithelial tumour of the pancreas. Other mesenchymal tumours that may occur in the pancreas include tumours of the peripheral nerve sheath, fibrous histiocytic tumours and rare vascular tumours.

12E7 Antigen↗