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Biomedical subjects

E Calonje

Publications and source records attributed to E Calonje.

104 records · Page 6Linked to original sources

Infantile hemangiopericytoma versus infantile myofibromatosis. Study of a series suggesting a continuous spectrum of infantile myofibroblastic lesions.

The clinicopathologic features of 11 tumors, originally diagnosed as infantile hemangiopericytomas and with a spectrum of morphologic findings, are described. The age of the patients ranged from 6 days to 7 years; seven patients were younger than 1 year (mean, 2.25 years; median, 10 months); six were boys and five were girls. Three neoplasms were situated in skin or subcutis and seven in deep soft tissue; in one case the depth was unstated. Seven lesions arose in the lower limbs, and one each in the lumbar region, clitoris, chest wall, and soft tissue of the zygomatic region. One patient later was found to have two additional dermal tumors, one each on the anterior abdominal wall and the chest wall. Follow-up information in eight patients revealed local recurrence 12 years later in one case only. Histologically, all tumors showed distinctive features of infantile hemangiopericytoma, including immature cytology, multilobulated growth pattern, focal necrosis, and mitotic activity in varying degrees. Vascular invasion was noted in seven cases. Additionally, a second tumor cell component, composed of spindle-shaped myofibroblastic cells forming fascicles and micronodules, was evident at least focally. Both the spindle cells and more primitive round cells were positive for alpha-smooth muscle actin. Both cellular components showed a haphazard zoning arrangement. We discuss the clinicopathologic similarities between infantile hemangiopericytoma and infantile myofibromatosis and point out the differences between infantile and adult hemangiopericytoma. Our study suggests that there exists a broad spectrum of benign infantile myofibroblastic lesions containing an immature-appearing cellular component with a distinctive, hemangiopericytoma-like vascular pattern. Infantile myofibromatosis and so-called infantile hemangiopericytoma almost certainly represent different stages of maturation of the same (single) entity.

Child↗

Epithelioid benign fibrous histiocytoma of skin: clinico-pathological analysis of 20 cases of a poorly known variant.

Twenty new cases of epithelioid fibrous histiocytoma, a distinctive but poorly recognized variant of cutaneous benign fibrous histiocytoma are described. Twelve patients were male and eight were female. The age of the patients ranged from 7 to 80 years (median 40 years). Six lesion arose on the lower limb, five on the upper limb, three around the shoulder, two on the abdomen and one each on the eyelid, inner canthus and anal margin. In one case the age and site were not stated. All lesions presented as a solitary, polypoid or slightly raised cutaneous nodule, ranging in size from 0.5 cm to 2 cm in greatest diameter. Follow-up in 14 cases (mean 22.7 months) revealed local recurrence after 42 months in one case. Histologically, the more polypoid tumours were characterized by an epidermal collarette. In all lesions prominent polygonal or rounded epithelioid cells with abundant eosinophilic cytoplasm, a vesicular nucleus and small eosinophilic nucleoli accounted for at least 50% of the tumour cell population. Individual cells were separated by somewhat hyalinized collagen, containing prominent blood vessels and a sparse mononuclear inflammatory cell infiltrate. The edge of the lesions, especially those that were non-polypoid, often showed more typical features of ordinary fibrous histiocytoma. The main differential diagnosis is with other cutaneous epithelioid neoplasms, especially Spitz naevus.

Adult↗

Cellular benign fibrous histiocytoma. Clinicopathologic analysis of 74 cases of a distinctive variant of cutaneous fibrous histiocytoma with frequent recurrence.

We report seventy-four cases of a distinctive variant of cutaneous fibrous histiocytoma, which is often mistaken histologically for sarcoma and which carries a high local recurrence rate. These tumors appeared most commonly in young or middle-aged adults, with a predominance in men (male/female ratio 1.9:1). Anatomic distribution was wide, with cases occurring mainly in the upper limb/limb girdle (34%), lower limb/limb girdle (27%), and head and neck region (20%). Most lesions had been present for only a few months, and their sizes ranged from 0.5 cm to 2.5 cm in maximum diameter. Twelve (26%) of 46 cases with follow-up (mean duration 3 years) recurred locally, in one case twice. Distinctive histologic features were a commonly fascicular growth pattern, predominance of eosinophilic spindle cells with tapering nuclei, a moderate mitotic rate (mean three per 10 high-power fields), and frequent extension into the subcutaneous fat (33% of cases). In addition, all cases showed at least focal cytologic polymorphism (inflammatory cells, foam cells, giant cells), and 58% showed associated epidermal alterations in common with usual cutaneous fibrous histiocytomas. Nine cases (12%) showed foci of central necrosis. Immunohistochemical studies (ABC method) found only vimentin and very focal smooth muscle actin positivity. Tests for CD34, desmin, S-100, keratin, and Factor XIIIa were negative in all cases. These lesions should be distinguished from dermatofibrosarcoma protuberans and leiomyosarcoma, with which many of these cases were initially confused.

Adolescent↗

Leiomyosarcoma with prominent osteoclast-like giant cells. Analysis of eight cases closely mimicking the so-called giant cell variant of malignant fibrous histiocytoma.

Eight cases of leiomyosarcoma with osteoclast-like giant cells, arising in deep soft tissue, and that mimicked closely the "giant cell variant of malignant fibrous histiocytoma (MFH)," have been studied morphologically and immunohistochemically. The age of the patients ranged from 7 to 88 years (mean, 66.2 years; median, 74 years); five were female patients. Three lesions arose in the lower limbs, two in the buttock, and one each in the shoulder, chest wall, and the floor of the mouth. Follow-up in one case revealed a local recurrence and in two cases systemic metastases. All cases showed, at least focally, interwoven spindle cell fascicles, with the cytologic features of smooth muscle cells, as well as strong positivity for alpha-smooth-muscle actin, muscle actin, and desmin. The morphologically benign osteoclast-like giant cells expressed CD68 but failed to stain with myogenic markers. The association of leiomyosarcoma with prominent osteoclast-like giant cells is not as uncommon as generally believed, being evident in 8.7% of the deep-seated nonvisceral leiomyosarcomas that we have studied. These results provide good evidence for myogenic differentiation in at least a subset of those tumors with morphologic features currently classified as the giant cell variant of MFH. Considering that at least some other reported cases of giant cell MFH appear to be a variant of extraskeletal osteosarcoma, we would suggest that lesions with this distinctive pattern should be more carefully classified according to their apparent line of differentiation.

Aged↗

Cutaneous crystalline deposits in myeloma.

BACKGROUND: Myeloma is a plasma cell malignancy that usually presents with systemic manifestations or symptoms related to bone involvement. We describe the first case of crystalline protein deposition in the skin as the initial manifestation of myeloma. OBSERVATIONS: Crystals were found mainly in the extracellular space in the dermis of both involved and uninvolved skin in the absence of plasma cell infiltration. Crystals were also found in conjunctival tissue and bone marrow. CONCLUSIONS: We have described a unique case of myeloma that presented as facial and eyelid swelling. There were crystalline deposits in the skin and conjunctivae, but mechanisms of crystal formation and the factors causing local deposition were not established. However, treatment of the underlying disorder leads to resolution of the cutaneous features of crystal deposition.

Biopsy↗

Retiform hemangioendothelioma. A distinctive form of low-grade angiosarcoma delineated in a series of 15 cases.

Fifteen cases of a distinctive type of low-grade angiosarcoma of the skin are described. Most tumors presented in the second to fourth decades of life, the youngest patient being 9 years old and the oldest 78 (mean age, 36 years). There was no sex predilection. Six tumors arose on the lower limb, four on the upper limb, three on the trunk, and one each on the penis and the scalp. One case arose in the setting of chronic lymphedema and another following radiotherapy for carcinoma of the uterine cervix. Distinctive morphologic features were the presence of long arborizing blood vessels arranged in a retiform pattern (reminiscent of normal rete testis) lined by monomorphic hobnail endothelial cells, a very prominent lymphocytic infiltrate in most cases, and the focal presence of papillae with hyaline collagenous cores, similar to those seen in malignant endovascular papillary angioendothelioma (Dabska's tumor). With a median follow-up of 7.25 years in 14 cases, retiform hemangioendothelioma has proved to be a low-grade neoplasm that recurs frequently but has a very low metastatic rate. The single regional lymph node metastasis in this series was from a case with a biphasic pattern in which only the spindle cell component was represented in the metastasis. There have been no tumor-related deaths, underlining the importance of accurate distinction from conventional angiosarcoma. This distinction is facilitated principally by the absence of dissection between individual collagen bundles and the absence of endothelial atypia or mitotic activity. The precise relationship between retiform hemangioendothelioma and Dabska's tumor is uncertain, possibly because cases of the latter may not be homogeneous.

Adolescent↗

Dermatomyofibroma: additional observations on a distinctive cutaneous myofibroblastic tumour with emphasis on differential diagnosis.

Following the very recent description of a benign plaque-like myofibroblastic tumour of the skin, known as dermatomyofibroma, we present four further cases, emphasizing its clinical features and its histological differential diagnosis. Three patients were adult females and one was a boy. Two of the lesions arose on the anterior abdominal wall, one on the shoulder, and one on the neck. Clinically, there was often close resemblance to a keloid. Follow-up revealed no evidence of recurrence after simple excision. Histologically, these were non-encapsulated but well-circumscribed, plaque-like lesions located in the reticular dermis, and composed of fascicles of monomorphic myofibroblasts, orientated parallel to the skin surface. Immunohistochemically, most of the tumour cells expressed alpha-smooth-muscle actin. The principal differential diagnosis is plaque-stage dermatofibrosarcoma protuberans. Other differential diagnoses include diffuse neurofibroma, dermatofibroma and other myofibroblastic skin tumours.

Actins↗

Spindle-cell non-pleomorphic atypical fibroxanthoma: analysis of a series and delineation of a distinctive variant.

Atypical fibroxanthoma is a bizarre, cytologically malignant but usually clinically benign, lesion which typically arises in sun-damaged skin of the head and neck region in the elderly. Classically, its morphology is said to represent the dermal counterpart of pleomorphic malignant fibrous histiocytoma. We have identified 10 cases of a more monomorphic spindle-celled, fascicular variant which, paradoxically, was often mistaken for a clinically malignant lesion because it lacked the pleomorphism of conventional atypical fibroxanthoma. These tumours all arose in the head and neck region as polypoid lesions in the elderly. The tumours were confined to the dermis, often had an epidermal collarette, showed an eosinophilic fascicular morphology and were highly mitotic. All 10 were vimentin positive and five showed very focal actin positivity. Desmin, keratin and S-100 protein were negative in all cases. The clinical course was benign in all cases, justifying their accurate recognition. The principal differential diagnoses are spindle cell squamous carcinoma, spindle cell melanoma and leiomyosarcoma. Immunohistochemistry plays a key role in this distinction.

Aged↗

Lipoblastoma and lipoblastomatosis: a clinicopathological study of 14 cases.

The clinicopathological features of 14 cases of lipoblastoma and lipoblastomatosis are presented. The age of the patients at presentation ranged from 5 days to 6 years (mean 2.7 years); nine patients were male. Histologically, six cases were circumscribed (lipoblastoma) while eight were diffuse and ill-defined (lipoblastomatosis). In both groups and in individual cases there was distinct lobulation, as well as a spectrum of adipocytic maturation. Cytologically, the 10 most mature lesions were composed of uniform adipocytes intermixed with only scattered lipoblasts and primitive mesenchymal cells. A notable feature in the other four cases was a prominent myxoid stroma producing a very close resemblance to myxoid liposarcoma. Mitotic figures were rare and always normal in appearance. Atypical nuclei were not evident. Follow-up in eight patients revealed local recurrence in two. Liposarcoma in patients under 10 years is exceedingly rare, and, in myxoid form, may be almost impossible to distinguish histologically from lipoblastoma. Helpful clues are the lack of lobulation, variable growth pattern and increased nuclear atypia in liposarcoma.

Child↗

Intravascular spread of keratoacanthoma. An alarming but benign phenomenon.

We report a patient with a keratoacanthoma of the scalp in which there was invasion of several medium-sized vessels by the tumour at a distance from the main lesion. A marked inflammatory response within the invaded vessels as well as a benign clinical course do not suggest that this phenomenon represents malignant transformation.

Blood Vessels↗

Cellular 'neurothekeoma': an epithelioid variant of pilar leiomyoma? Morphological and immunohistochemical analysis of a series.

Cellular neurothekeoma is a recently recognized benign cutaneous neoplasm, which is currently regarded as being of nerve sheath origin and is thought to represent a variant of conventional neurothekeoma (dermal nerve sheath myxoma). Nine new cases presenting predominantly in adolescents or young adults are described. Morphologically they were characterized by short fascicles or small nests of palely eosinophilic epithelioid or spindle-shaped cells which ramified in an ill-defined manner between dermal collagen bundles. Myxoid matrix was absent or sparse. Scattered normal mitoses and multinucleate giant cells were often present. Immunohistochemically all nine cases were strongly NK1/C3 positive, seven were weakly NSE positive and three were smooth muscle actin positive. Staining for S-100 protein, PGP 9.5, epithelial membrane antigen and desmin was negative in all cases. In view of its distinctive architecture and immunophenotype, both of which are totally different from conventional neurothekeoma, it is proposed that cellular 'neurothekeoma' is a separate discrete entity which may represent an epithelioid variant of pilar leiomyoma.

Adolescent↗

Sinusoidal hemangioma. A distinctive benign vascular neoplasm within the group of cavernous hemangiomas.

Twelve cases of sinusoidal hemangioma, a distinctive subset of the group of lesions known as cavernous hemangioma, are described. All presented as solitary subcutaneous/deep dermal lesions in adults, predominantly females. Five arose on a limb and five on the trunk; two of the latter were situated in mammary subcutaneous tissue. Histologically they were characterized by dilated, interconnecting, thin-walled vascular channels that frequently showed a pseudopapillary pattern. These vessels had a predominantly lobular architecture but peripherally showed focally ill-defined spread into subcutaneous tissue. The lining endothelium was single-layered but showed focal pleomorphism and hyperchromasia, which, combined with the pseudopapillae and apparent infiltrative pattern in areas, raised the possibility of angiosarcoma in four cases, most notably in the breast lesions. This possibility was further suggested by the presence of pseudonecrotic central infarction in two cases. Follow-up in eight cases, however, has revealed no tendency for either local recurrence or metastasis.

Adult↗

Irritant threshold and histological response of epidermis to irritant application.

Individuals vary in their ability to react to irritants, which can be demonstrated for sodium lauryl sulfate (SLS) using the irritant threshold (IT) test. We aimed to study whether the histological and immunohistochemical features of the skin following SLS exposure varied with subject's IT. 8 subjects were recruited. Their IT was measured. Biopsies were taken after 2 hr and 4 hr of occlusion with 20% SLS and control. The specimens were stained with haematoxylin and eosin and for Langerhans cells. At 4-hr, low-threshold subjects developed changes to a greater extent than high-threshold subjects. The relationship of histological reaction to IT could be related to a differential pro-inflammatory cytokine response in subjects. Low IT has been previously associated with a tumour necrosis factor alpha promoter region polymorphism.

Antigens, CD1↗

New entities in cutaneous soft tissue tumours.

Connective tissue tumours represent a significant group among biopsied skin lesions and manifest a wide range of morphological patterns. While the majority are easily diagnosed, notably most fibrous histiocytomas and neural lesions, a proportion pose considerable diagnostic difficulty. With the increasing recognition and characterisation of previously undescribed entities within the family of soft tissue tumours in recent years, the number of unclassifiable lesions should diminish. The ultimate aim of accurate recognition and classification is to provide appropriate clinical care based on reasoned prediction of a given lesion's behaviour. With this in mind, we wish to briefly review herein some of the more recently characterised dermal connective tissue lesions, including plexiform fibrohistiocytic tumour, cellular fibrous histiocytoma, spindle celled atypical fibroxanthoma, adult myofibroma, dermatomyofibroma, so-called cellular neurothekeoma, ectopic meningothelial hamartoma, tufted angioma, sinusoidal haemangioma and bacillary angiomatosis.

Adult↗