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Biomedical subjects

E Calonje

Publications and source records attributed to E Calonje.

At least 91 records · Page 5Linked to original sources

Epithelioid hemangioendothelioma of skin and soft tissues: clinicopathologic and immunohistochemical study of 30 cases.

Epithelioid hemangioendothelioma of soft tissues (EHE) represents a distinct entity with an unpredictable clinical course. We analyzed the clinicopathologic and immunohistochemical features in a series of 30 patients. Patient age range was 16-74 years (median 50); 18 of 30 patients were female. Eight tumors arose in the lower and two in the upper extremities, seven on the trunk, five each in the head/ neck and anogenital regions, two in the mediastinum, and one in the abdomen. Seventeen neoplasms were located in deep soft tissues, nine were subcutaneous or perifascial, and four were dermal; size ranged from 0.4 to 10 cm; in 11 cases the tumor was > 5 cm. Tumors with an infiltrative growth pattern were more common than entirely circumscribed lesions. The tumors were composed histologically of short strands, cords, or small clusters of epithelioid, round, to slightly spindled endothelial cells that formed at least focally, intracellular lumina and were set in a frequently myxohyaline stroma. Thirteen of 30 lesions showed angiocentric growth, which was occlusive in many cases. Immunohistochemically, all cases tested were positive for at least one endothelial marker (CD31, CD34, factor VIII, Ulex europaeus), six of 23 (26%) were positive for cytokeratin, and five of 11 (45%) were positive for alpha-smooth muscle actin. Median follow-up of 36 months (range 2-96) in 24 cases showed local recurrence in three cases and systemic metastases in five cases (21%); four patients (17%) died of tumor. Although more aggressive histologic features (striking nuclear atypia in eight cases, numerous spindled cells in 10, more than two mitoses per 10 high-power fields in nine, and small, more solid angiosarcomalike foci in four cases) tended to be related to poor clinical outcome, there was no clear correlation. Two metastasizing cases showed no histologically atypical features whatever. We suggest that EHE of soft tissue is better regarded as a fully malignant, rather than borderline, vascular neoplasm, albeit the prognosis is better than in conventional angiosarcoma.

Adolescent↗

Squamous cell carcinoma arising in chronic lymphoedema.

Two patients are reported in whom a squamous cell carcinoma arose in chronic lymphoedematous skin on the lower legs. Although lymphangiosarcoma is a well recognized complication of chronic lymphoedema, squamous cell carcinoma has only been reported on seven previous occasions. The pathogenesis of malignant tumours in chronic lymphoedema is discussed.

Carcinoma, Squamous Cell↗

[Pigmented fibrosarcomatous dermatofibrosarcoma protuberans (Bednar tumor). 3 case reports, analogy with the "conventional" type and review of the literature].

BACKGROUND: The so-called Bednar tumor represents a pigmented variant of dermatofibrosarcoma protuberans (DFSP) and is characterized by a usually scant (1-5% of cells) population of dendritic melanocytes within an otherwise typical DFSP. This pigmented variant accounts for up to 5% of all DFSPs. Other variants of DFSP include cases showing features (in the primary or recurrence) of either giant cell fibroblastoma or fibrosarcoma. Less than 5% of DFSPs are associated with metastases and many of these show either a fibrosarcomatous component or, much more rarely, an "MFH"-like appearance. Only one previous case has been reported which showed combined features of the pigmented and fibrosarcomatous variants. MATERIALS: We present herein 3 cases of fibrosarcomatous Bednar tumor, all occurring in males, 2 aged 75 and 1 aged 23; two patients were white and one black. The tumors were located on the trunk or shoulder and two had been present for many years with recent rapid growth. One patient developed local recurrence and metastases to bone and lung and died within 1 year. The other two patients are disease free at 3 and 5 years follow-up respectively. All three cases showed typical histological features and in two tumors the pigment was evident macroscopically. CONCLUSIONS: A through literature review, including all cases of fibrosarcomatous DFSP and metastasizing fibrosarcomatous DFSP (whether or not pigmented), confirms that the fibrosarcomatous variant of DFSP (including its pigmented counterpart) is significantly more aggressive than usual DFSP, thus underlining the importance of its accurate recognition.

Adult↗

Myxofibrosarcoma. Clinicopathologic analysis of 75 cases with emphasis on the low-grade variant.

Myxofibrosarcoma is one of the most common sarcomas in the extremities of elderly patients. We analysed the clinicopathologic features in a series of 75 patients. All patients were adults (range, 22-91 years; median, 66 years) with an approximately equal incidence in men and women. Thirty-five tumors arose in the lower and 25 in the upper extremities, nine on the trunk, two each in the retroperitoneum and the head and neck region, and one each in the pelvis and penis. Forty-eight cases (69.5%) were located in dermal or subcutaneous tissues. Distinctive histologic features included the following: a commonly nodular growth pattern; a myxoid matrix containing elongated, curvilinear capillaries; and fusiform, round or stellate tumor cells with indistinct cell margins, slightly eosinophilic cytoplasm, and hyperchromatic atypical nuclei. These lesions varied from a hypocellular, mainly myxoid, and purely spindle-cell appearance (low-grade neoplasms) to high-grade, pleomorphic (malignant fibrous histiocytoma-like) lesions with multinucleated giant cells, high mitotic activity, and areas of necrosis. Immunohistochemistry in 44 cases revealed only vimentin and occasional actin positivity. Ultrastructurally, tumor cells had a fibroblastic phenotype. DNA flow cytometry and proliferation analysis showed an association between aneuploidy and histologic grade. An average follow-up of 45 months (range, 5-300 months) in 60 cases has revealed local recurrence in 33 cases (54%). Thirteen patients developed metastases, and 13 tumor-related deaths occurred. A short interval to first local recurrence was associated with poor clinical outcome. The rate of local recurrence was independent of histologic grade, but only intermediate and high-grade neoplasms metastasized. The depth of the primary lesion did not influence the incidence of local recurrence. However, in deep-seated neoplasms, the incidence of metastases was higher and the percentage of tumor-related deaths was twice as high as in superficially located lesions, reflecting the fact that deep-seated lesions tended to be higher-grade, larger tumors. Myxofibrosarcoma tends to become progressively higher grade in recurrences, as demonstrated in five cases in our series. The poorly recognized low-grade myxofibrosarcoma is emphasized, as proper diagnosis and treatment and scrupulous follow-up are mandatory to avoid local recurrence and gradual tumor progression to a higher-grade neoplasm that may then metastasize.

Adult↗

Myoid differentiation in dermatofibrosarcoma protuberans and its fibrosarcomatous variant: clinicopathologic analysis of 5 cases.

We report a series of five cases of dermatofibrosarcoma protuberans, four of which were fibrosarcomatous and all of which showed histologic and immunohistochemical evidence of focal myoid/myofibroblastic differentiation (accounting for up to 5% of each tumor). These lesions were identified amongst 208 cases of dermatofibrosarcoma protuberans including 24 examples of the fibrosarcomatous variant. Three of the five patients were male and two were females; all were adults (37-72 years). One case arose on the scalp and two cases each on the abdominal wall and upper trunk. All tumors were less than 5cm in diameter and preoperative duration ranged from 2 months to 10 years. In three cases with follow-up there was no recurrence. Histologically, all tumors were typical fibrosarcomatous or ordinary dermatofibrosarcoma protuberans but for the presence of scattered to confluent nodules and bundles of eosinophilic spindle cells associated with well-defined cytoplasmic margins and vesicular nuclei associated with focal stromal hyalinization. While the typical dermatofibrosarcoma protuberans areas were CD34 positive, the myoid areas were negative for this antibody and positive for smooth muscle actin and pan-muscle actin. All tumors were desmin negative. Recognition of myofibroblastic differentiation in fibrosarcomatous dermatofibrosarcoma protuberans is important not only because it gives support to the theory of a fibroblastic/myofibroblastic line of differentiation for this type of tumor, but also because it might be a source of confusion with other myofibroblastic lesions (e.g. myofibromatosis, adult myofibroma), especially when small biopsies are evaluated.

Adult↗

Fibrolipomatous hamartoma of nerve arising in the brachial plexus.

Fibrolipomatous hamartoma of nerve is a rare benign infiltrating condition of peripheral nerves leading to progressive functional loss. Previous descriptions have virtually all been confined to the distal part of the upper limb, with the median nerve being the commonest reported site. Most cases occur in the first 3 decades of life and a third are associated with macrodactyly. A case is described in a 63-year-old woman, involving the whole of the brachial plexus, a previously unreported site for this particular lesion.

Biopsy↗

Monophasic cellular variant of infantile myofibromatosis. An unusual histopathologic pattern in two siblings.

Infantile myofibromatosis (IMF) is a distinct clinicopathologic entity characterized by solitary or multicentric tumors present at birth or in early infancy with a typical biphasic (central hemangiopericytoma-like, peripheral leiomyoma-like) histologic pattern. We present a case of IMF in two siblings with onset of disease in late childhood. Histology of the primary as well as several later tumors was characterized by a monophasic cellular appearance with a prominence of tiny capillaries initially suggesting an unusual vascular tumor. Diagnosis was established by the development of more characteristic biphasic lesions during the course of disease. Immunocytochemistry (Ulex, factor VIII, JC/70A [CD31], PAL-E, BMA120, EN4, QBEnd10 [CD34], SMS actin) and ultrastructural studies showed no (marked) differences between different types of IMF. The monophasic cellular pattern should be recognized as an unusual histologic manifestation of IMF, in particular in patients outside the classical setting or presentation.

Actins↗

Cutaneous intraneural glomus tumor.

The unusual occurrence of a glomus tumor within a small, deep dermal nerve is reported. This phenomenon has been described previously only once in the literature. The lesion appeared as a painful cutaneous nodule in the shoulder of a 67-year-old woman. Microscopically, a typical solid, smooth muscle actin-positive glomus tumor was seen, encircled completely by neural tissue in the deep dermis. There was no extension of the tumor beyond the nerve. Immunohistochemically, the nerve fibers surrounding and within the tumor were positive for S-100 protein and neurofilament. The presence of perineurium surrounding the glomus tumor was confirmed by a layer of epithelial membrane antigen-positive cells. In view of the absence of normal glomus bodies in the nerves, an origin from a less specialized perivascular cell is proposed.

Actins↗

Lymphangiomatosis of the limbs. Clinicopathologic analysis of a series with a good prognosis.

Six cases of a distinctive but poorly recognized variant of lymphangiomatosis with predominant or exclusive involvement of the soft tissues of the limb/limb girdle are described. The six patients were male and presented with slowly progressive swelling of the involved limb. The age of onset was at birth (three cases), 3 months (one case), 11 years (one case) and 12 years (one case). Five patients had involvement of the lower extremity and one of the right upper extremity. Three patients had concomitant asymptomatic bone involvement either in the affected limb (two cases) or in distant bones (one case). Only one patient had visceral involvement that was limited to the ipsilateral thorax and was associated with chylothorax. Diagnosis was confirmed in all but one patient by lymphangiography. Treatment consisted principally of surgical reduction with significant clinical improvement. No patient later developed systemic involvement and the clinical course was benign. The bone lesions did not progress in any patient. Histologically, each case was characterised by interconnecting, dilated lymphatic spaces, lined by a single, attenuated layer of endothelial cells, involving the dermis, subcutis, and occasionally, underlying fascia and skeletal muscle with characteristic and extensive "dissection" of collagen and surrounding normal adnexal structures. Despite the absence of red blood cells in the vascular spaces, interstitial hemosiderin deposition was prominent in four cases. As opposed to most cases of lymphangiomatosis, which usually have extensive visceral involvement associated with a very poor prognosis, involvement in this variant is limited almost exclusively to soft tissues of the limb and bone and is associated with good prognosis.

Arm↗

Aneurysmal benign fibrous histiocytoma: clinicopathological analysis of 40 cases of a tumour frequently misdiagnosed as a vascular neoplasm.

Forty cases of the distinctive but poorly recognized aneurysmal variant of cutaneous fibrous histiocytoma are described. These tumours presented most commonly in middle-age adults, with a slight predilection for females. Anatomical distribution was wide with most cases occurring in the lower limb/limb girdle (50%), upper limb/limb girdle (20%) and trunk (17%). Lesional size ranged from 0.5 cm to 4 cm. Haemorrhage accounted for the rapid clinical growth of some lesions and the frequent clinical confusion with a cyst, a melanocytic lesion or a haemangioma. Five (19%) of the twenty-six cases with follow-up (mean duration 2.5 years) recurred locally, twice in two cases. One of these cases had involvement of a regional lymph node in the second recurrence, most likely as a result of direct local extension. Distinctive histological features were prominent blood-filled spaces, varying from artefact-like clefts to cystic areas mimicking cavernous vascular channels but devoid of an endothelial lining, prominent haemosiderin deposition, numerous siderophages and giant cells, and a moderate mitotic rate. Despite the presence of prominent secondary changes due to haemorrhage, all cases showed cellular polymorphism, hyalinized collagen bundles surrounded by tumour cells in the periphery of the lesion and 88% showed some degree of epidermal hyperplasia, as seen in common fibrous histiocytoma. Immunohistochemistry (ABC method) revealed only vimentin and, rarely, focal smooth muscle actin positivity. CD68 was positive in some reactive macrophages only. Stains for CD31, CD34, desmin and factor XIIIa were negative in all cases tested.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Inflammatory leiomyosarcoma: a morphological subgroup within the heterogeneous family of so-called inflammatory malignant fibrous histiocytoma.

Twelve cases of inflammatory leiomyosarcoma are presented. These tumours arose in the deep soft tissues of the trunk and proximal limbs. The age of the patients ranged from 13-53 years (median 36 years); there was an approximately equal sex ratio. Follow-up data was available for nine patients (mean duration 3.3 years); local recurrence occurred in three and lung metastases in one. Lesions were spindle cell neoplasms with fascicular areas which occupied between 5% and 80% of the tumour. Areas with a distinct storiform pattern were also seen in 10 cases. A prominent inflammatory cell component was evident in all tumours, often masking the neoplastic spindle cells. Histiocytes were identified in all cases, with aggregates of xanthoma cells seen in eight tumours. In 10 cases there was also a dense lymphoid infiltrate and in two a marked polymorphonuclear leukocyte infiltrate was evident. Immunohistochemistry showed in all tumours that the spindle cells stained positively for myogenic markers (8 of 12 positive for desmin, 10 of 12 for alpha smooth muscle actin and 11 of 12 for HHF-35). CD68 was expressed by the histiocytic infiltrates. Many of these tumours were diagnosed initially as inflammatory malignant fibrous histiocytoma. We provide evidence that at least one subset of neoplasms, which would have been formerly classified under this rubric, in fact show smooth muscle differentiation. Further studies are required to investigate the possibility that other tumour types or lines of differentiation may be present within the category of so-called inflammatory malignant fibrous histiocytoma.

Adolescent↗

Pseudomalignant perineurial invasion in cellular ('infantile') capillary haemangiomas.

One hundred and sixty-eight cellular ('infantile') capillary haemangiomas were assessed for the presence of perineurial invasion, a feature that can lead to the erroneous diagnosis of malignancy. Fourteen tumours (8%) showed unequivocal, usually prominent, involvement of small and medium sized nerves. Eleven of these lesions presented in infants at birth or shortly thereafter, two in young adults and one in a middle-aged adult. Ten patients were females. Ten of the lesions arose in the head and neck region, one in the arm, one in the chest wall, and in two the site was not stated. Follow-up revealed a local recurrence in only one of six cases. Histologically, all cases were typical pure capillary haemangiomas composed of lobules of small blood vessels, lined by bland endothelial cells, involving the dermis, subcutis or both. One case was an intramuscular capillary haemangioma. Long-standing cases, especially those in adults, were less cellular, with focal fibrosis and a myxoid stroma. Neural invasion was detected either in the centre or at the periphery of tumour lobules and was characterized by the presence of variable numbers of capillaries within the perineurium and in close contact with Schwann cells. This feature was highlighted by immunostaining for S-100 protein and EMA. In one case, extensive invasion of medium-sized deep dermal veins was also present, focally simulating an intravascular pyogenic granuloma. This study demonstrates that perineurial invasion in infantile capillary haemangiomas is a relatively common finding and should not be regarded as evidence of malignancy.

Adolescent↗

Giant cell angiofibroma. A distinctive orbital tumor in adults.

A series of seven cases of a previously unrecognized potentially recurrent tumor occurring in the orbit of adult patients is reported. This lesion shows histologic appearances intermediate between, but distinct from, solitary fibrous tumor and giant cell fibroblastoma of soft tissue. Morphologically it is characterised by a richly vascularized, patternless spindle-cell proliferation containing pseudovascular spaces. Multinucleate giant cells (often of floret type) and cells with large, rounded nuclei are present both in the cellular areas and also lining the pseudovascular spaces. The stroma is variably collagenized or sometimes myxoid. Immunohistochemically, the tumor cells exhibit positivity for vimentin and CD34. Follow-up in five cases (median duration 24 months) revealed local recurrence in one patient and persistent tumor in another. The clinical and morphologic features enable distinction of this lesion from both solitary fibrous tumor and giant cell fibroblastoma, and we suggest the designation "giant cell angiofibroma of the orbit".

Adult↗

Immunohistochemistry and DNA flow cytometry in soft-tissue sarcomas.

Immunohistochemistry has a major role as an adjunct to light microscopy in the diagnosis and classification of soft tissue sarcomas. Immunohistochemical analysis of proliferation markers appears so far to add very little to accurate and experienced histologic typing of soft-tissue sarcomas. Immunohistochemical assessment of tumor suppressor genes and oncogene products gives an insight into pathogenetic mechanisms but does not appear to correlate reliably with clinical outcome. Analysis of ploidy by DNA flow cytometry does not separate benign from malignant lesions, and assessment of cell proliferation by this method does not seem to predict clinical behavior accurately.

Biomarkers, Tumor↗

[Vascular tumors of the skin and soft tissue. Overview of newly characterized entities and variants].

This review summarizes vascular tumours of skin and soft tissues that have been characterised in recent years. Although most of them are very rare, knowledge about their reproducible clinicopathological features is important to avoid diagnostic pitfalls. These lesions include: bacillary angiomatosis, a vasoproliferative, pseudoneoplastic infection of immunocompromised patients caused by Rochalimaea henselae; tufted angioma, a variant of lobular capillary haemangioma characterised by a "cannon-ball" distribution of multiple lobules composed of packed capillaries and pericytes; microvenular haemangioma, a cutaneous haemangioma composed of thin-walled and irregularly branching blood vessels which dissect dermal collagen; sinusoidal haemangioma, a distinctive variant of cavernous haemangioma which may be located in the subcutaneous breast tissue and then may be confused with well-differentiated angiosarcoma; "hobnail haemangioma" (targetoid haemosiderotic haemangioma), a benign vascular tumour with a distinctive clinical targetoid appearance and a hobnail cytomorphology of the prominent endothelial tumour cells; retiform haemangioendothelioma, a very recently characterised low-grade angiosarcoma occurring most commonly in the extremities of adolescents which is characterised by arborising blood vessels arranged in a retiform pattern and lined by hobnail-like prominent endothelial cells; Kaposi-like infantile haemangioendothelioma, a borderline malignant tumour of infants mimicking Kaposi's sarcoma histologically; epithelioid angiosarcoma, a highly aggressive tumour in the spectrum of epithelioid vascular lesions which stains positively for endothelial and epithelial immunohistological markers; benign lymph-angioendothelioma (progressive lymphangioma), a benign, slowly growing macule or plaque which has to be distinguished from well-differentiated angiosarcoma and Kaposi's sarcoma; and lymphangiomatosis of the limbs, a poorly recognised angiomatosis occurring in young patients and limited mainly to the limbs with a favourable prognosis.

Connective Tissue↗