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Biomedical subjects

B Zelger

Publications and source records attributed to B Zelger.

At least 55 records · Page 3Linked to original sources
PubMed1998

Cellular 'neurothekeoma': an epithelioid variant of dermatofibroma?

AIMS: Cellular neurothekeoma is a rare benign cutaneous neoplasm with conflicting opinions regarding its histogenetic origin (nerve sheath, smooth muscle, myofibroblasts) as well as its relation to myxoid neurothekeoma (nerve sheath myxoma). The present series describes 15 cases whose clinicopathological features indicate a relationship to dermatofibroma. METHODS AND RESULTS: In this retrospective clinicopathological study, the lesions preferentially occurred in adolescents to young adults on the upper half of the body, often clinically diagnosed as some kind of fibrohistiocytic tissue response. Besides characteristic whorled nests to fascicles of palely eosinophilic epithelioid cells, all lesions showed variable clues pointing to dermatofibroma: acanthosis, ill-defined storiform periphery, peripherally accentuated prominent sclerosis and lymphocytic demarcation/infiltration. Immunohistochemically, all cases were positive with NK1C3 (CD57), KiM1p and proliferating cell nuclear antigen, seven were positive for neurone specific enolase, five for factor XIIIa, six for smooth muscle specific actin and three for E9, an antimetallothionein marker. These findings are similar to those of conventional dermatofibromas, the variability of the profile being best explained by time cycle and function dependent changes. Ultrastructurally, two cases showed microfilaments, attachment plaques, prominent pinocytosis and focal remnants of basal lamina. A careful study of the data and photomicrographs from the literature reveals that in many cases similar conclusions could be reached. Obvious discrepancies are most likely due to the confusion with myxoid neurothekeoma, a well circumscribed, more spindly and myxoid, S100 positive lesion of Schwannian origin. CONCLUSION: The appearance of dermatofibromas is markedly influenced by architectural, e.g. in deep penetrating dermatofibroma, and/or cellular/stromal criteria, e.g. in epithelioid cell histiocytoma or sclerosing dermatofibroma. Cellular neurothekeoma seems to be a variant of dermatofibromas with both architectural and cellular/stromal peculiarities, i.e. plexiform pattern, epithelioid cytology and stromal sclerosis.

Actins↗
PubMed1998

An unusual case of deep penetrating dermatofibroma of the lower leg. Case report.

Histological examination of a punch biopsy specimen of a slowly growing, irregular dermal tumour from the right lower leg of a 32-year-old woman, which had been diagnosed clinically as a dermatofibrosarcoma protuberans, showed it to be a deep penetrating dermatofibroma. The diagnosis was established by characteristic histological and immunohistochemical criteria. This allowed sequential excision in two sessions with primary closure and an optimal cosmetic result.

Adult↗
PubMed1998

[Cellular "neurothekeoma": an epithelioid variant of dermatofibroma?].

AIMS: The present series describes 15 cellular neurothekeomas whose clinicopathologic features indicate a close relationship to dermatofibroma. METHODS: Retrospective clinicopathologic study. RESULTS: Lesions preferentially occurred in adolescents to young adults on the upper half of the body, often clinically diagnosed as some kind of fibrohistiocytic tissue response. Besides characteristic whorled nests to fascicles of palely eosinophilic epithelioid cells all lesions showed variable clues pointing to dermatofibroma: acanthosis, ill-defined storiform periphery, peripherally accentuated prominent sclerosis and lymphocytic demarcation/infiltration. All cases were positive with NK1C3 (CD 57), Ki-M1p and proliferating cell nuclear antigen, 7 for neuron specific enolase, 5 for factor XIIIa, 6 for smooth muscle actin and 3 with E9, an anti-metallothionein marker. These findings are similar to other types of dermatofibromas, the variability of the profile being best explained by time cycle and function dependent changes. Ultrastructurally, two cases showed microfilaments, attachment plaques, prominent pinocytosis and focal remnants of basal lamina. A careful study of data and microphotographs from the literature reveals that in many cases similar conclusions can be reached. Obvious discrepancies are most likely due to the confusion with myxoid neurothekeoma, a well circumscribed, more spindly and myxoid, S 100 positive lesion of Schwannian origin. CONCLUSION: According to our results cellular neurothekeoma seems to be a whorled-nested to plexiform epithelioid variant of dermatofibroma.

Adult↗
PubMed1998

[Fibrohistiocytic skin lesions].

AIMS: A new unifying concept of fibrohistiocytic skin lesions. METHODS: Retrospective clinicopathologic study of more than 2,000 dermatofibromas (DF) recruited over the last two decades. RESULTS: As the least common denominator all DF show a reactive fibrohistiocytic tissue response with variable epidermal hyperplasia, peripheral sclerosis and peripherally accentuated lymphohistiocytic tissue response. The histological, immunohistochemical and ultrastructural profile varies according to the time cycle as well as the fibro-myofibrohistiocytic differentiation of the lesions. From a conceptual point of view such lesions are best grouped into three categories: 1. DF with cellular/stromal pecularities. All classic variants, from the first description by Unna in 1894 to lesions described in 1978, namely DF, (benign) fibrous histiocytoma, histiocytoma cutis, sclerosing hemangioma, nodular subepidermal fibrosis or fibrous xanthoma, fall into this category; moreover, many of the clinicopathologic variants described over the last two decades such as granular cell, clear cell, myofibroblastic, sclerosing, monster cells, atypical ("pseudosarcomatous"), haemosiderotic ("elusive"), cholesterotic, and myxoid variants. 2. DF with architectural pecularities such as deep penetrating, atrophic, aneurysmal ("angiomatoid"), haemangiopericytoma-like, palisading and ossifying variants. And 3. DF with both cellular/stromal and architectural pecularities including those with a homogenous mixture of components as seen in epithelioid, cellular benign variants, smooth muscle proliferation in DF, multinucleate cell angiohistiocytoma, cellular neurothekeoma, and dermal plexiform fibrohistiocytic tumour; as well as those with heterogeneous mixture of components in composite or mixed dermatofibromas. CONCLUSION: DF is common, the clinicopathological variability manyfold, and their misinterpretation as malignancy such as dermatofibrosarcoma protuberans or Kaposi sarcoma not rare. Such cases have important clinical implications such as unnecessary investigations, controls and livelong anxiety of patients.

Histiocytoma, Benign Fibrous↗
PubMed1997

Metallothionein overexpression in human brain tumours.

Metallothioneins (MTs) are metal binding proteins overexpressed in various human neoplasms which are associated with resistance to cytotoxic drugs. A series of 156 archival human brain tumours were investigated immunohistochemically for expression of MTs; these included 10 low-grade gliomas, 44 high-grade gliomas, 98 meningeal tumours (19 classical, 30 atypical, 38 anaplastic meningiomas, and 11 haemangiopericytomas or papillary meningiomas), and 4 other tumours. Low-grade gliomas showed heterogeneous MT expression; 32 high-grade gliomas (72.7%) showed MT expression of more than 25% of tumour cells without statistically significant differences between first operations and recurrent tumours. In 2 glioblastomas, the presence of MT was confirmed by Western blotting. The extent of MT immunoexpression showed a statistically significant inverse relationship to the degree of p53 immunoreactivity. In meningiomas, a tendency to a higher percentage of MT-expressing cells was observed from classical over atypical to anaplastic meningiomas, but these differences were not statistically significant. In conclusion, MT expression is present in a significant portion of, especially malignant, brain tumours and might be involved in their poor response to antineoplastic drugs.

Adult↗
PubMed1997

Sclerotic lipoma: lipomas simulating sclerotic fibroma.

AIMS: We report a series of five unusual subcutaneous lipomas characterized by prominent stromal sclerosis in a storiform arrangement. METHODS AND RESULTS: All lesions occurred in young male adults (23.0 +/- 9.5 years), three lesions were on the scalp and two on the hands. Clinically, lipoma, chondroma or some form of cyst were suspected. In contrast to sclerotic fibroma ('circumscribed storiform collagenoma') a variable number of adipocytes were interspersed within the fibrosclerotic background. These cells also showed immunoreactivity for S100 protein as well as intracytoplasmic lipid deposits ultrastructurally. None of the lesions recurred within a follow-up period of between 0.5 and 9 years. CONCLUSIONS: Our series suggests that sclerotic lipomas should be classified with fibro- and myxolipomas, angiolipomas, spindle cell and pleomorphic lipomas, as an additional characteristic subtype of lipoma. Sclerotic lipomas are often misdiagnosed as a sclerotic fibroma, but are essentially fatty tumours and are, at least in our small series, not associated with Cowden's disease.

Adolescent↗
PubMed1997

Granular cell dermatofibroma.

AIMS: To describe a series of five granular cell dermatofibromas as an unusual and rare manifestation of fibrohistiocytic tissue response. METHODS AND RESULTS: Five granular cell dermatofibromas were collected out of 136 tumours filed as granular cell tumours. Clinically, all lesions occurred on the shoulder or back of middle-aged adults (two women, three men), mostly with the clinical diagnosis of a fibrohistiocytic lesion. Histology revealed well-circumscribed, dermal to subcutaneous lesions dominated by periodic acid-Schiff (PAS) positive, granular cells. Acanthosis above, as well as storiform arrangement of spindle cells, sclerotic collagen and some interspersed lymphohistiocytic infiltrate at the periphery of the lesion, indicated the fibrohistiocytic origin. Lesions showed prominent reactivity with NK1C3 (CD57), as well as for macrophage markers KiM1p and KP1 (CD68). In contrast to classic Schwannian/neurogenic granular cell tumours, granular cell dermatofibromas were S100 protein negative, but showed variable reactivity for factor XIIIa (10-50%) in 4/5, for smooth muscle specific actin (10-50%) in 2/5 and with E9 (10-30%) in 3/5 lesions. Electron microscopy in one case revealed large pools of phago-lysosomes and variably sized glycogen granules in granular cells. CONCLUSION: Our series delineates granular cell dermatofibroma as a distinct clinicopathological variant of fibrohistiocytic tissue response which needs to be distinguished from other tumours with granular cell features.

Adult↗
PubMed1997

Clear cell dermatofibroma.

This series presents six cases of a rare variant of dermatofibroma, characterized by marked clear cell change. All lesions occurred on the lower extremities of middle-aged adults (four women, two men), mostly with the clinical diagnosis of fibrohistiocytic lesion. Histological examination revealed well circumscribed, faintly stained dermal to subcutaneous lesions which were due to the overwhelming presence of clear cells (> 90%), some with prominent PAS-positive cytoplasmic granulation. Overlying epidermal hyperplasia as well as storiform arrangement of spindle cells, sclerotic collagen and some interspersed lympho-histiocytic infiltrate at the periphery of the lesion indicated the fibrohistiocytic origin. Individual histopathological peculiarities included: bizarre giant cells in two cases, perifollicular arrangement and haemangiopericytoma-like features with iron deposition in one case each. Immunohistochemically three of four lesions showed moderate reactivity for factor XIIIa and two of four with an anti-metallothionen marker E9, but were otherwise negative with a broad panel of markers. Electronmicroscopy in two cases revealed large pools of glycogen beside focal, prominent endoplasmic reticulum and lysosomes in some granular cells, but only optically translucent cells in cases of clear cells. Recognition of clear cell dermatofibroma is important as the differential diagnosis includes some entities with more serious outcome/considerations such as metastases of renal cell carcinoma, xanthogranulomatous reactions, balloon cell naevus/melanoma and clear cell sarcoma.

Adult↗
PubMed1997

[Isolated extracranial meningioma of the facial soft tissues].

BACKGROUND: Isolated primarily extracranial meningiomas are extremely rare tumors of the head and neck region. The pathogenesis of these benign lesions is not fully clarified. Most likely these tumors derive from ectopic meningeal or arachnoid cells following the nerve sheaths of cranial nerves along their course through the skull base. PATIENT: We report on a patient presenting with a slow growing tumor of the soft facial tissue. The tumor was resected completely via an external approach. RESULTS AND CONCLUSION: The patient has been free of tumor recurrence for one year. The pathogenesis of the extracranial meningioma in this patient seems to be the displacement of meningeal cells in the course of a fracture of the base of the skull.

Adipose Tissue↗
PubMed1997

Verocay body--prominent cutaneous schwannoma.

We report on eight cases of a distinct variant of cutaneous schwannoma characterized by prominent Verocay body formation (75-100% of the tumor bulk) that may cause considerable diagnostic difficulties. Like ordinary cutaneous schwannomas, these lesions preferred the head and neck region of young adults without sexual predilection and were clinically interpreted as cyst, basal cell carcinoma, or nevus. Histological examination revealed well-circumscribed nodules. Three of them consisted exclusively of nodular or ribbon-like Verocay bodies. A variable admixture of Antoni A or B type of differentiation (< 25%) was seen in five other cases. The following patterns were seen: fascicular spindle-shaped, onion-like epithelioid, myxoid-hypocellular, and degenerated ("ancient") with prominent fibrosis/hyalinosis and occasional bizarre giant cells. Immunohistochemically, the lesions were positive for S-100 protein (and vimentin) but negative for a broad panel of neurogenic and intermediate filament markers. The capsule showed focal labeling for EMA and--when it was markedly thickened--also for SMA. Labeling with E9, an anti-metallothionein marker indicative of cell activity, was negative, underscoring the slow growth potential of these lesions. No recurrence was seen in the six patients with follow-up information. The differential diagnosis includes other lesions with prominent palisading. (Amianthoid) myofibroblastoma and palisading leiomyoma are consistently positive for SMA and desmin, respectively. Palisading cutaneous fibrous histiocytoma and myofibroblastic dermatofibroma are variably positive for Factor XIIIa, SMA, and E9 and/or NK1C3 (CD57). Palisaded encapsulated neuromas are primarilly differentiated by the presence of nerve fibers with myelin sheaths.

Adolescent↗
PubMed1997

Dermal and subcutaneous variants of plexiform fibrohistiocytic tumor.

We report five cases of plexiform fibrohistiocytic tumors, three classic subcutaneous lesions and two dermal ones. Both variants had similar profiles and were clinically indistinguishable. The lesions affected the trunk more than the upper extremities and were found in children and young adults (18.4 +/- 12.8 years). They showed a marked female predominance (4:1, including both dermal variants). Clinically, they were skin-colored, hard nodules of 1-2-cm diameter that resulted in such differential diagnoses as fibroma, histiocytoma, pilomatricoma, or cyst. Interestingly, one subcutaneous case with a painful "worms in the sack" presentation was thought to represent a plexiform neurofibroma. Histology revealed well-circumscribed dermal or subcutaneous plexiform lesions with a characteristic biphasic appearance. Most of the tumor bulk consisted of spindle-shaped to stellate myofibroblasts with a variable admixture of collagen or loosening of stroma. In the center of the plexiform strands and nodules, a few osteoclast-like giant cells as well as epithelioid mononuclear cells (< 10%) were found. Myofibroblasts were positive with HHF35 and for smooth muscle actin in three of five cases. Osteoclast-like giant cells were positive with KP1 in all five cases. Both types of cells stained with the macrophage marker Ki-M1p. A broad panel of other markers was negative. This series expands the spectrum of plexiform fibrohistiocytic tumor, but it also broadens the differential diagnosis of (dermal) plexiform lesions, which at present includes spindle cell nevi, schwannomas, neurofibromas, granular cell tumors, nerve sheath myxomas (neurothekeomas), spindle cell lipomas, and tufted angiomas.

Adult↗
PubMed1997

Human papillomavirus DNA in non-melanoma skin cancers of a renal transplant recipient: detection of a new sequence related to epidermodysplasia verruciformis associated types.

The detection of human papillomavirus (HPV) types originally isolated from patients with epidermodysplasia verruciformis (EV) in skin tumors of transplant recipients may point to a role of this HPV subgroup in non-melanoma skin cancer in immunosuppressed people. We analyzed 17 formalin-fixed, paraffin-embedded biopsies of benign or malignant skin tumors of a renal transplant patient with unusually widespread cutaneous carcinomas. Using a nested polymerase chain reaction (PCR), HPV-specific DNA was demonstrated in 11 specimens (65%). Analysis of nine PCR amplification products revealed four different sequences related to EV-associated HPVs. Three sequences occurred only in one lesion. In six samples identical sequences were found that differed from all HPV sequences published to date and may therefore represent a novel EV-HPV type, preliminarily labeled RTRX7. RTRX7 was found in benign, premalignant, and malignant skin lesions. Alignments identified HPV12 as the closest relative of RTRX7, both in the DNA (81% homology) and in the amino acid sequence (84% homology).

Adult↗
PubMed1997

Malignant melanomas simulating various types of soft tissue tumors.

BACKGROUND: Primary malignant melanomas, recurrences, and metastases thereof can present with a wide variety of clinicopathologic aspects. OBJECTIVE: The present series describes eight primary malignant melanomas and/or 10 local recurrences/metastases (from eight patients) misdiagnosed as various types of soft tissue tumors: two dermatofibrosarcoma protuberans, two atypical fibroxanthomas, two storiform-pleomorphic malignant fibrous histiocytomas, one myxofibrosarcoma ("myxoid malignant fibrous histiocytoma"), two malignant hemangiopericytomas, and nine malignant schwannomas. METHODS: In three cases correct diagnosis was established by clinicopathologic correlation during follow-up; all the others were only discovered during a retrospective work-up of all soft tissue tumors diagnosed at the Dermatohistopathological Laboratory of the Department of Dermatology, University of Innsbruck. RESULTS: Helpful clues derived from subtle intraepidermal, irregular spread of melanocytes, focal nested arrangement of tumor cells, sparse melanin deposition, neurotropism, and prominent peripheral lymphohistiocytic response, partially forming lymph follicles. Besides clinicopathologic correlation, histology of serial sections as well as immunohistochemistry (S100 protein more important than NK1/C3; HMB45 without benefit) and electron microscopy (melanosomes) proved helpful for definitive diagnosis. In contrast to the general assumption that spindle cell/desmoplastic malignant melanomas have an unequivocal bad prognosis, our series, as well as evidence from the literature, document a better prognosis than that of other types with comparable tumor thickness (70% vs 50% 5-year survival). Moreover, labeling with E9, an antimetallothionein marker, confirmed its usefulness for prognosis being strongly positive in primary lesions followed by rapid progression, but mostly negative in those without. CONCLUSION: These cases document that malignant melanoma may mimic various types of soft tissue tumors; correct interpretation is important as prognosis and therapeutic management differ considerably between these entities.

Aged↗
PubMed1997

Cutaneous epithelioid hemangioendothelioma, epithelioid cell histiocytoma and Spitz nevus. Three separate epithelioid tumors in one patient.

We report the unusual history of a young lady who, over a period of 9 years, developed 3 epithelioid neoplasms of different histiogenetic origin: a cutaneous epithelioid hemangioendothelioma, an epithelioid cell histiocytoma, and a Spitz nevus. To the best of our knowledge, such an unusual combination of neoplasms has not been reported previously and raises the question of an underlying "epithelioid" reaction pattern responsible for the epithelioid appearance in all 3 tumors; the likelihood of such a combination of tumors occurring in a single patient would be less than 1 in 1 billion.

Adult↗
PubMed1996

Expression of a glycoprotein of the carcinoembryonic antigen family in normal and neoplastic sebaceous glands. Limited role of carcinoembryonic antigen as a sweat gland marker.

BACKGROUND: Carcinoembryonic antigen (CEA) is a well-known marker for sweat gland differentiation in adnexal neoplasms. OBJECTIVE: The aim of this study was to examine the expression of glycoproteins of the CEA family, that is, CEA-180, nonspecific cross-reacting antigens (NCAs), and biliary glycoprotein (BGP), in sebaceous glands and in neoplasms with sebaceous differentiation. METHODS: Normal adult and fetal skin, hyperplasias, hamartomas, and neoplasms with sebaceous or follicular differentiation were stained immunohistochemically with a panel of polyclonal and monoclonal antibodies highly specific for CEA-180, NCAs, and BGP. Double immunostaining was performed to correlate the CEA expression with that of epithelial membrane antigen (EMA), a glycoprotein consistently found in differentiating sebocytes. RESULTS: Whereas sweat glands coexpressed CEA, NCAs, BGP, and EMA, sebaceous glands were exclusively labeled with the antibodies recognizing BGP or EMA. Staining of the sebaceous glands was restricted to mature sebocytes, sparing immature cells. At the ultrastructural level immunoreactivity for BGP and EMA was demonstrable in the golgi area, in small vesicles, and along the cell membranes. During fetal development BGP was not found until the sebaceous glands matured. The expression of BGP and EMA was highly conserved in reactive, hamartomatous, and neoplastic proliferations of adnexal structures with sebaceous differentiation. CONCLUSION: The expression of BGP, a CEA glycoprotein, in differentiating sebocytes accounts for the reactivity of many anti-CEA antibodies with sebaceous glands and thus disqualifies the CEA family as a monospecific marker for sweat gland differentiation.

Adolescent↗