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Biomedical subjects

B G Zelger

Publications and source records attributed to B G Zelger.

At least 19 recordsLinked to original sources
PubMed2005

A new variant of mastocytosis: report of three cases clinicopathologically mimicking histiocytic and vasculitic disorders.

Cutaneous mastocytosis (CM) or urticaria pigmentosa is characterized by abnormal proliferation and accumulation of mast cells. Clinically, CM usually presents as symmetrically distributed red-brown macules or papules that develop weals, erythema and often pruritus on stroking (Darier's sign). The histological hallmark of the disease is an increase in oval to spindle-shaped mast cells in the dermis located around blood vessels and skin appendages. We describe three patients with a new clinicopathological type of CM, which clinically mimics a histiocytic disorder and histologically mimics leucocytoclastic vasculitis (LV). Three infants (two boys and one girl) developed generalized reddish-yellow-brown macules of 3-10 cm with occasional scaling and crusting on the trunk and extremities without further symptoms or organ involvement except variable itching. Histology revealed diffuse and dense dermal infiltrates of eosinophils, neutrophils and nuclear debris with perivascular accentuation, imitating LV. This infiltrate masked large epithelioid cells, positive for macrophage markers, which by special histochemical stains for metachromatic granules turned out to be mast cells. This is the first report of this new variant of CM, which may cause considerable diagnostic difficulties both clinically and histopathologically.

Adult↗
PubMed2004

[Epithelioid sarcoma of the hand, a seldomly recognized tumor].

BACKGROUND: The epithelioid sarcoma of the hand is often misdiagnosed. This tumor occurs most frequently in children and young adults, has an uncharacteristic course, and carries a high risk for local recurrence and metastases. The characteristic histopathology is helpful in establishing the correct diagnosis. Differential diagnosis includes synovialoma, fibromatosis and Dupuytren's contracture. Multinodular appearance is not uncommon. PATIENTS AND METHOD: Three young patients (one male and two female) were operated on the hand for treatment of Dupuytren's contracture, synovialoma and fibromas, respectively. The necessity for surgical revision in these patients led to a reevaluation of the primary histological data. The original diagnosis having been found to be incorrect, the correct diagnosis was established namely, epithelioid sarcoma. RESULTS: Surgical revision was performed, which involved partial amputation of the extremity. None of the patients underwent adjuvant radio- or chemotherapy. The patients were followed-up for ten, four and two and a half years, respectively. They are currently free of recurrences. CONCLUSION: Prognosis depends on the size and the location of the tumor. Adequate treatment requires early radical excision; amputation may be required if the primary tumor is located in the fingers or treatment of recurrent growth. The tumor tends to metastasize via the lymphatic system. Patients must be monitored with yearly lung X-rays since recurrence or metastasis may occur many years after the initial diagnosis and treatment.

Adult↗
PubMed2002

[Pathological examination of breast biopsy specimens].

No differences for long-term disease free survival could be found between breast conserving surgery and mastectomy. Most importantly is the fact that this therapy presents a significantly higher risk for local recurrence. The characterisation of this risk is one of the most important things to do. These findings result in a widespread change in treatment of breast cancer patients. Consequently an increase in interdisciplinary working between radiologists, surgeons and pathologists could be found. Histological examinations are necessary for diagnosis and exactly evaluation of the tumor extension. Microscopic evaluation of the resection margin is of most important interest, because there is a direct connection between local recurrence and tumor infiltration of the resection margin. We performed our investigations by the use of a standardized complete embedding method with the possibility of three-dimensional reconstruction, on a cohort of 280 patients. Additionally this method allowed the detection of all relevant findings on one hand and on the other hand an evaluation of all resection margins. Our results showed a breast conserving therapy including tumor free margins was performed in 67% of the patients. But there was a second resection necessary in 57% of the cases. An extensive tumor distribution as the detection of multifocal tumor spread was the reason for mastectomy in 33%. Our findings point out the necessarily of the histological examination in the line of the complete embedding method of the breast biopsy material in order to analyse the tumor including resection margin evaluation.

Adult↗
PubMed2000

Combined dermatofibroma: co-existence of two or more variant patterns in a single lesion.

AIMS: Based on a series of 25 cases, we define and characterize combined dermatofibroma, a tumour comprising two or more variant patterns of dermatofibroma in a single lesion. METHOD AND RESULTS: Dermatofibroma may present with a wide variety of architectural, cellular or stromal peculiarities. Architectural peculiarities include deep penetration, atrophy, collarette formation, fascicular to plexiform architecture, massive haemorrhage, prominent haemangiopericytoma-like vascularity and palisading; cellular peculiarities the presence of epithelioid cells, clear cells, granular cells, prominent myofibroblastic differentiation and atypical giant cells ('monster cells'); or stromal peculiarities such as prominent sclerosis, mucin, haemosiderin and cholesterotic deposits. In combined dermatofibromas two or more of these features are seen in complex or inhomogenous combination such as the silhouette of a deep penetrating dermatofibroma with an 'ordinary' storiform pattern in the upper and granular cell differentiation in the lower part of the lesion; or a dermatofibroma with ordinary features in the upper, prominent sclerosis in the middle and clear cells in the lower portion of the lesion; or the characteristic epidermal collarette and cells of epithelial cell histiocytoma with a plexiform ('neurothekeoma-like') architecture surrounded by a myxoid stroma with spindle-shaped to stellate cells. Clinically, these lesions preferentially occur on the lower extremities of young to middle-aged females, frequently with the diagnosis of a fibrohistiocytic lesion. Apart from one recurrence follow-up was uneventful in all other cases. Immunohistochemically, lesions are consistently positive with KiM1p, variably positive for factor XIIIa, smooth muscle specific actin and with KP1 (CD68), NK1C3 and E9. CONCLUSION: Recognition of combined dermatofibroma allows the histopathologist to apply a confident benign label to unusual lesions which might otherwise elude diagnosis, or tempt description of 'new' entities and to avoid a misdiagnosis of malignancy.

Actins↗
PubMed1999

[Intestinal invagination due to endometriosis of the terminal ileum].

HISTORY AND CLINICAL FINDINGS: A 39 year old female patient presented with nausea and crampy abdominal pain in the right lower quadrant. INVESTIGATIONS: Physical examination showed a markedly distended abdomen with a diffuse pain, punctum maximum in the right lower part of the abdomen and reduced peristalsis. Plain abdominal x-rays revealed slightly dilated loops of small bowel with air-fluid levels. Abdominal ultrasound revealed thickening of the bowel wall of the distal ileum and some free fluid. To exclude Crohn's disease, Sellink-computer tomography was performed, which also showed thickening of the wall of the terminal ileum and dilated bowel loops proximal to the underlying stenosis. DIAGNOSIS, TREATMENT AND COURSE: Intraoperatively, the terminal ileum was thickened by scar tissue and subtotally invaginated. Histologic investigation of the resected specimen showed submucosal endometriosis. CONCLUSION: Endometriosis of the small bowel should be considered carefully in the differential diagnosis of female patients of reproductive age who suffer from symptoms of ileus, dysmenorrhea and sterility.

Adult↗
PubMed1999

Myxoid dermatofibroma.

AIMS: This report presents seven cases of a rare variant of dermatofibroma, characterized by marked mucinous stromal changes. METHODS AND RESULTS: In this retrospective clinicopathological study, lesions preferentially occurred on the lower extremities of young to middle-aged females. Clinical (differential) diagnoses included: dermatofibroma (two cases), cyst, tophus, ganglion and neurofibroma. Histology revealed well circumscribed, faintly stained dermal to subcutaneous lesions which contained abundant stromal mucin. A variable amount of spindle-shaped cells, occasional lymphocytes and fine collagen fibres were also seen. Epidermal hyperplasia above as well as storiform arrangement of spindle cells and some sclerotic collagen at the periphery of the lesion indicated the fibrohistiocytic origin. Individual histopathological features included: bizarre giant cells, perifollicular arrangement and seas of mucin with pseudovascular clefts in one case each. Immunohistochemically, all lesions were positive for KiM1p (> 50% of the cells). Moreover, two lesions showed moderate reactivity for factor XIIIa (10-25%), one each with NK1C3 (> 90%) and smooth muscle actin (50%), but were otherwise negative with a broad panel of markers. Electron microscopy in one case revealed large pools of mucin interspersed by a moderate number of fibrocytes with fine fibrillary collagen. CONCLUSION: This series confirms myxoid dermatofibroma as a rare variant of fibrohistiocytic tissue response; our series also indicates that many of these lesions might be misinterpreted as cutaneous myxoma.

Adult↗
PubMed1999

Differential expression of CD34 and Ki-M1p in pleomorphic fibroma and dermatofibroma with monster cells.

Pleomorphic fibroma (PF) and dermatofibroma with monster cells (DFMC) are characterized by the presence of numerous cells with large atypical nuclei. Despite cytologic similarities, the two entities are likely to be unrelated, but their histogenesis is poorly understood. In this study, we examined six cases of PF and eleven cases of DFMC by immunohistochemistry using antibodies against vimentin, alpha-smooth muscle actin, S-100 protein, CD34, factor XIIIa, and the pan-monocytic marker Ki-M1p. Strong vimentin expression was seen in all tumors, whereas none of them expressed S-100 protein. PF consistently exhibited CD34 staining but appeared to be depleted of Ki-M1p positive cells compared with the surrounding normal skin. Conversely, all cases of DFMC contained numerous Ki-M1p positive cells including atypical multinucleate cells, but virtually no CD34 reactivity was observed. A weak staining for alpha-smooth muscle actin was occasionally seen in a subset of the cells of both entities. Our results indicate that PF and DFMC are histogenetically distinct entities that may arise from two different types of dermal dendritic cells defined by their reactivity for CD34 and Ki-M1p, respectively. Immunohistochemistry using these two antibodies permits an easy and reliable discrimination between PF and DFMC.

Actins↗
PubMed1998

[Dermatofibroma. A clinico-pathologic classification scheme].

Dermatofibroma is a common benign fibrohistiocytic lesion which presents with a wide variety of clinicopathologic variants. This may cause great difficulties in delineation from a variety of benign and malignant tumours. According to their peculiarities we differentiate: 1. Dermatofibromas with architectural peculiarities like deep penetrating, atrophic, giant, aneurysmal ("angiomatoid"), haemangiopericytoma-like, palisading or ossifying variants. 2. Dermatofibromas with cellular/stromal peculiarities like clear cell, granular cell, myofibroblastic, sclerotic, monster cell, atypical ("pseudosarcomatous"), elusive ("haemosiderotic"), cholesterotic, and myxoid variants. 3. Dermatofibromas with architectural and cellular/stromal peculiarities in homogenous arrangement like epithelioid cell, cellular benign variants, with smooth muscle proliferation, basal cell carcinoma-like, pseudolymphomatous, multinucleate cell angiohistiocytoma, cellular neurothekeoma, plexiform fibrohistiocytic tumour, plexiform xanthoma and plexiform xanthomatous tumour. 4. Complex or composite dermatofibromas with two or more architectural and cellular/stromal peculiarities in inhomogenous arrangement, e.g. silhouette of an epithelioid cell histiocytoma with plexiform fascicles of cellular neurothekeoma and granular cell features.

Histiocytoma, Benign Fibrous↗
PubMed1998

Scalloped cell xanthogranuloma.

AIMS: We describe nine cases of scalloped cell xanthogranuloma, a distinct solitary variant of non-Langerhans cell histiocytoses. METHODS AND RESULTS: In this retrospective clinicopathological study scalloped cell xanthogranuloma mostly occurred on the back or head and neck of young adult males diagnosed as a xanthogranuloma, naevus, or basal cell carcinoma. Histology characteristically revealed a sheet-like infiltrate of predominantly scalloped histiocytes (> 79% of all cell types) in the upper dermis. Other mononuclear (vacuolated, xanthomatized, spindle-shaped, oncocytic) and multinucleate (foreign body, ground glass and Touton) histiocytes were also regularly seen. Immunohistochemically, all cases exhibited a macrophage/dendritic cell lineage positive with KP1 (CD68), KiM1p, HAM 56 and factor XIIIa. Ultrastructurally, numerous intracytoplasmic dense, occasionally also myeloid bodies were present. No associated systemic disease, hyperlipidaemia or recurrence were seen during follow-up. CONCLUSIONS: These findings are similar to those of early lesions of xanthoma disseminatum: thus, scalloped cell xanthogranuloma could be regarded as a solitary counterpart of xanthoma disseminatum, and, moreover, fits neatly into a unifying concept of non-Langerhans cell histiocytoses.

Adolescent↗
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

[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↗