Are dermatologists greedy?
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to D J Santa Cruz.
Explore the source record for details and available documents.
The majority of cutaneous angiosarcomas display typical architectural features of irregular anastomosing vascular channels in the dermis and subcutis. Nuclei are usually hyperchromatic and pleomorphic but the volume of cytoplasm of the neoplastic cells is often small. Diagnosis can be made readily on an adequate biopsy. We recently experienced difficulty diagnosing an angiosarcoma composed predominantly of cells with abundant granular cytoplasm. We were able to compare the present case with sections obtained from the only other reported example. The architectural expression of an anastomosing vascular pattern in areas of tumor, combined with the positive staining for Factor VIII-related antigen (FVIIIRAg) and Ulex europaeus agglutinin-1 (UEA1) enabled us to make a diagnosis of angiosarcoma. The tumor failed to stain for the other endothelial markers (CD31 and CD34) which were positive in the original case. A marker for lysosomes (CD68) stained the granules in both cases. The granular cell variant of cutaneous angiosarcoma is very rare. Diagnosis is possible by recognizing the typical anastomosing neoplastic vascular channels at the periphery of the lesion, and by use of a combination of lectin (UEA1) and immunohistochemical (FVIIIRAg, CD34 and CD31) endothelial markers.
Epithelioid cell histiocytoma is a recently recognized lesion that is considered to be a variant of cutaneous fibrous histiocytoma (dermatofibroma). Ten cases are presented, including their light microscopic, immunohistochemical, and ultrastructural features. Eight of the cases are similar to those previously reported, presenting as elevated nodules arising on the extremities and composed of epithelioid histiocytes with overlying epidermal effacement. Two of the cases were composed of cells with the same morphologic and immunohistochemical characteristics as typical epithelioid cell histiocytoma, including factor XIIIa positivity, but these arose in the reticular dermis and exhibited prominent cellularity.
Explore the source record for details and available documents.
Clear cell basal cell carcinoma (BCC) is an unusual variant of BCC which is characterized by a variable component of large clear cells. Twenty cases are presented which are further subdivided into pure, mixed, or metatypical categories. Although initially thought to represent sebaceous or tricholemmal differentiation, electron microscopic studies demonstrated that the clear cell changes are a degenerative phenomenon, probably involving lysosomes.
BACKGROUND: Angioendotheliomatosis has recently been separated into two types. Malignant angioendotheliomatosis is a lymphoma in which the neoplastic cells accumulate in vascular lumens. Reactive angioendotheliomatosis is a proliferation of capillaries that is less well characterized. OBJECTIVE: Our purpose was to describe the clinical and histopathologic features of three cases of reactive angiomatosis in patients with cryoproteinemia. METHODS: Clinical and histologic data were correlated. Immunoperoxidase staining and electron microscopy were used in the histologic evaluation. RESULTS: All three cases showed tufts of capillaries with luminal deposits of cryoproteins. Immunohistochemical studies showed that the cellularity of the tufts was primarily caused by a proliferation of pericytes. CONCLUSION: Cryoproteinemia can cause angiomatosis that simulates a vascular neoplasm.
Eight cases of apocrine (tubular branching lumina) type cutaneous mixed tumors with follicular and sebaceous differentiation are presented. All eight tumors arose on facial skin; six patients were male and two were female. The lesions showed a cystic or nodular clinical appearance and were surgically excised. Histopathological examination confirmed the diagnosis of apocrine type of cutaneous mixed tumor in each case. Follicular differentiation consisted of (a) keratinous cysts with infundibular keratinization (infundibular differentiation); (b) hair bulbs with papillary mesenchyma, matricial differentiation with basophilic, transitional, and shadow cells, trichohyaline granules, vellous hair shafts, and clear cells of the outer root sheath (anagen differentiation); and (c) epithelial columns composed of inner cells with plump oval nuclei and scant cytoplasm, and similar cells at the periphery that were arranged in a palisade, resembling the inferior segment of a normal hair follicle in telogen. Sebaceous differentiation was represented by mature sebaceous cells, either as single cells or as small islands, within epithelial tracts of the tumor. The proportion of the areas showing these different types of differentiation varied among lesions, but some follicular differentiation was always present, whereas three cases lacked sebaceous differentiation. Immunohistochemical analysis in three cases with respect to their eccrine or apocrine differentiation showed contradictory results as in a previously reported series of cutaneous mixed tumors. The presence of follicular and sebaceous differentiation in the apocrine (tubular branching lumina) type of cutaneous mixed tumor is a confirmation of the apocrine nature of this neoplasm as well as an expression of the common embryologic derivation of all elements of the folliculosebaceous-apocrine unit.
Six cases of a distinct, histologically anaplastic variant of mammary Paget's disease are described. Patients ranged in age from 40 to 85 years. All patients had scaling erythematous lesions confined to the nipple; none had palpable breast masses. Histologically, the lesions had features resembling Bowen's disease, including full-thickness epidermal atypia, loss of nuclear polarity, and marked cytologic anaplasia. Intraepidermal acantholysis was a distinctive feature in all cases. In some biopsies, small groups and single typical Pagetoid cells were seen within the areas of confluent Bowen-like change. Immunohistochemically, carcinoembryonic antigen (CEA) was positive in three of six patients; epithelial membrane antigen (EMA) in five of six patients, and cytokeratin AE1/AE3 in three of six patients. Mucicarmine stains were uniformly negative. In our series, anaplastic Paget's disease was associated with concomitant invasive ductal carcinoma in three of six patients (50%). This percentage is significantly higher than that previously reported for patients with Paget's disease and without palpable breast mass. Histologic features that are helpful in distinguishing between anaplastic Paget's disease and Bowen's include cleft-like acantholysis, absence of dyskeratotic cells, and persistence of basal cell layer. More rarely, but very helpful when present, are underlying ductal carcinoma, intracellular lumina, and associated conventional Paget's disease. Immunohistochemistry results were variable and of relative value. Our study suggests that a nipple lesion histologically resembling Bowen's disease is likely to represent anaplastic Paget's disease.
We describe two patients with crusted perineal plaques that were biopsied and diagnosed as Paget's disease. Resection specimens of each contained a dermal mucinous carcinoma with extensive epidermotropism and coexistent epidermal basaloid proliferations closely resembling fibroepithelioma (Pinkus). The presence of the Paget phenomenon was supported by histochemical, immunohistochemical, and ultrastructural evidence. No other primary neoplasms were found in either patient. Followup at 2 1/2 and 3 1/2 years, respectively, has been negative. We conclude that either the fibroepitheliomatous changes may be induced by or may represent a collison (unlikely) with the epidermotropic mucinous carcinoma. It is proposed that the concept fibroepitheliomatous Paget phenomenon be used to stand for the histologic changes common to our cases as well as those previously reported.
Explore the source record for details and available documents.
In recent years, the classification of vascular lesions has been expanded and modified with the addition of several newly described entities, the redefinition of others, the recognition of lesions of borderline biologic behavior, and the need to avoid misdiagnosis with early Kaposi's sarcoma. This review clarifies the nomenclature, updates information on previously known lesions, and summarizes data on several recently discovered, lesser-known entities such as glomeruloid hemangioma, microvenular hemangioma, and multinucleate cell angiohistiocytoma. Clinicopathologic features and differential diagnosis are emphasized.
Thirteen cases of a unique cutaneous tumor are presented. The lesions presented as single, nondescript, skin-color nodules. Eleven were located on the head and two were on the legs. The male/female ratio was 8:5. The age at diagnosis ranged between 21 and 55 years (mean: 39). The duration of the lesions was from several months to more than 20 years. All tumors were excised and did not recur. The tumors typically presented as well-circumscribed nodules with scant or no epidermal connections. (One example, however, had a plate-like configuration amply connected with the basal layers of the epidermis.) The proliferation consisted of multiple, rounded lobules of basaloid cells with some degree of peripheral palisading, immersed in a dense, fibrous stroma. There was an intense infiltrate of small lymphocytes within the lobules, with some spillage into the stroma. No clear adnexal differentiation was noted except for rare isolated cells showing apparent sebaceous differentiation within the tumor lobules. Areas of central keratinization were also present. Numerous cells with ample amphophilic cytoplasm, large vesicular nuclei, and prominent nucleoli were also seen. Immunohistochemistry confirmed the presence of keratin within tumor cells. Common leukocytic antigen highlighted the intense intralobular lymphocytic component. Vimentin not only highlighted the stromal fibroblastic component, but also stained scattered intralobular cells. Epithelial membrane antigen was positive within some of the large intraepithelial cells. S-100 protein was extensively positive within dendritic intralobular and stromal cells. Lymphocytic markers demonstrated a polyclonal B and T population. This unique tumor appears to represent a form of adnexal neoplasm with basaloid features, possibly immature pilosebaceous differentiation.
Juvenile xanthogranuloma occurring in soft tissue is rare and has received little attention. This report describes cases of deep juvenile xanthogranuloma occurring in the soft tissues of three children. Each tumor was a solitary lesion that arose, respectively, in the superficial skeletal muscles of an 8-month-old girl, the subcutis of the scalp of a 3-month-old boy, and the subcutis of the forehead of a 10-year-old girl. Two lesions were grossly firm, tan-yellow, and homogeneous. Histologically, the subcutaneous lesions were relatively circumscribed; the third lesion infiltrated muscle and contained widely separated skeletal muscle fibers. All lesions showed sheets of uniform amphophilic or acidophilic cells with occasional eosinophils and rare Touton giant cells. In two cases and in cutaneous controls, positive immunoperoxidase stains (HAM-56, HHF-35, and vimentin) supported macrophagic-myofibroblastic differentiation. S-100 protein, MAC-387, and factor XIIIa were negative. Electron microscopy in one case also supported macrophagic-myofibroblastic differentiation. Langerhans granules were absent. Follow-up of 7, 6, and 5 years indicated no recurrences. The differential diagnosis includes deep fibrous histiocytoma and cellular subcutaneous neural tumors.
Ten examples of a distinctive vascular tumor are reported. These benign acquired lesions typically occur as small, enlarging lesions that favor the extremities, particularly the forearms, of young to middle-aged adults. Clinically, they are purple to red lesions generally thought to be hemangiomas. Histologically, there is a pattern of irregular, branching venules with inconspicuous lumina and lack of cellular atypia. Because the lesions do not conform to existing classifications of vascular tumors, they have been designated with the histologically descriptive name of microvenular hemangioma. Although speculative, they are felt to represent a form of acquired venous hemangioma.
A cutaneous mastocytoma with associated histologic features of eosinophilic cellulitis is reported. The tumor occurred as a small, asymptomatic lesion on the left thigh of a 4-year-old boy. Microscopically, an accumulation of mast cells, microgranulomas, eosinophils and 'flame figures' was present. A pathogenesis involving mast cell degranulation, eosinophil chemotactic factors and eosinophil major basic protein is discussed.
Four cases of large eccrine acrospiroma (three benign, one malignant) are reported. The benign tumors involved the lower extremities of two women and one man (73 to 89 years of age). The duration of the lesions ranged from 10 to 20 years. The malignant tumor involved the left side of the chest of a 60-year-old man. Its occurrence in a lesion that had been present for 40 years suggested malignant transformation of a pre-existing benign eccrine acrospiroma. Each tumor showed little to no cellular atypia. Mitotic rates (mitotic figures per 10 high-power fields) varied both between and within lesions. Average mitotic rates did not differentiate the benign from the malignant tumors. The most important distinguishing features of large benign eccrine acrospiromas are the relative circumscription, the lack of cellular atypia, and the absence of stromal, perineurial, and angiolymphatic invasion.