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D J Santa Cruz

Publications and source records attributed to D J Santa Cruz.

At least 19 recordsLinked to original sources

Angiomatosis with luminal cryoprotein deposition.

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.

Aged

Apocrine type of cutaneous mixed tumor with follicular and sebaceous differentiation.

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.

Adenoma, Sweat Gland

Anaplastic Paget's disease.

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.

Adult

Fibroepithelioma-like changes associated with anogenital epidermotropic mucinous carcinoma. Fibroepitheliomatous Paget phenomenon.

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.

Adenocarcinoma, Mucinous

Acquired benign and "borderline" vascular lesions.

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.

Angiokeratoma

Cutaneous lymphadenoma.

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.

Adult

Deep juvenile xanthogranuloma. Subcutaneous and intramuscular forms.

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.

Child

Microvenular hemangioma.

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.

Adolescent

Eosinophilic cellulitis: histologic features in a cutaneous mastocytoma.

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.

Cellulitis

Giant eccrine acrospiroma.

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.

Adenoma, Sweat Gland

Cellular angiolipoma.

Three examples of cellular angiolipoma are presented. They occurred as part of multiple, occasionally painful, subcutaneous nodules on the extremities and trunk of healthy men. There was no familial tendency. Grossly, the cellular angiolipomas were small lesions, approximately 1 cm. Histologically, dense cellular angiomatous tissue comprised more than 95% of the lesions. Much of this component contained prominent spindle cells. Lesser degrees of involvement in the other subcutaneous nodules illustrated the continuum of histology between cellular angiolipomas and more typical angiolipomas. The differential diagnosis of cellular angiolipomas includes spindle cell lipoma, Kaposi's sarcoma, and other vascular tumors. The most important distinguishing features are encapsulation, intravascular fibrin thrombi, septation, association with other more typical angiolipomas, and occurrence in healthy individuals.

Adult

Desmoplastic trichilemmoma: histologic variant resembling invasive carcinoma.

A clinical and histologic review of 22 patients (13 males, 9 females) with cutaneous lesions classified as desmoplastic trichilemmoma is reported. Typically, the lesions occur as solitary dome-shaped papules on the face during (at least) the fifth decade of life. The average age was 64 years (range 19-89) with a median age of 66.5 years. The most frequent clinical diagnosis was basal cell carcinoma, and the most common sites were the lip, eyebrow and nose. Two lesions occurred in examples of nevus sebaceus of Jaddasohn. The proportion of desmoplasia varies, but is generally between 20% to 60% of the lesion. Ulceration is seen in a minority of lesions but when present, is closely associated with underlying desmoplasia. Desmoplasia may be seen in small, as well as large, trichilemmomas; it generally occurs centrally but at times may be seen peripherally. The appearance of strands of epithelial cells entrapped in dense fibrosis and hyalinization may mimic desmoplastic variants of squamous cell carcinoma and basal cell carcinoma. This appearance may be particularly troublesome when the desmoplasia occurs at the base of the biopsy specimen. Knowledge of this phenomenon and the search for more typical features of trichilemmoma such as clear cells and peripherally palisaded columnar cells upon a thickened, eosinophilic basement membrane will allow the correct diagnosis to be made. We believe that the desmoplasia occurs as a secondary change in pre-existing trichilemmomas.

Adult

Cholesterotic fibrous histiocytoma. Its association with hyperlipoproteinemia.

A 63-year-old woman with fibrous histiocytomas showed cholesterol deposition in the setting of type IIB hyperlipidemia. The two lesions involved the left leg and right thigh. One had typical features of a fibrous histiocytoma including changes of the overlying epidermis. The other was essentially replaced by cholesterol deposits and could not be differentiated from a tuberous xanthoma. This case illustrates the histiocytic response of fibrous histiocytomas to a hyperlipoproteinemic microenvironment.

Cholesterol

Cutaneous inflammatory pseudotumor. Lesions resembling "inflammatory pseudotumors" or "plasma cell granulomas" of extracutaneous sites.

This report describes four cases of a previously undocumented circumscribed idiopathic inflammatory fibrosclerotic lesion of the skin. The lesions occurred in two black women and two white men; they had been apparent from months to a year. The nodules were solitary; two were located on the arm, one on the calf, and one on the posterior neck. The clinical diagnoses included nodules, keratinous cyst, pilar tumor, pilomatricoma, vascular leiomyoma, dermatofibroma, and metastatic carcinoma. The lesions were grossly white, homogeneous, and circumscribed; one was located in the superficial subcutis and three in the reticular dermis. Histologically, they were sharply circumscribed and surrounded by a dense peripheral rim of lymphocytes and plasma cells. Peripheral germinal centers were noted in two cases. All cases had variable amounts of fibrosis centrally, giving them the low-power appearance of lymph nodes; however, no subcapsular or medullary sinuses were found. In the center of the lesions, the mononuclear infiltrate was accompanied by eosinophils and neutrophils. Virtually imperceptible vasculature was noted in portions of three lesions; in the fourth lesion, high endothelial venules were seen in the lymphoid portions, and "targetoid" perivascular sclerosis was seen in the central portions. There appears to be a transition from the early, mostly inflammatory, lesions to the sclerotic ones. The process seems to be reactive in nature. No obvious local or systemic etiology was found in our series, and follow-up of 2 to 5 years has been noncontributory. These mixed-cell proliferations resemble the extracutaneous masses that have been described in numerous viscera--particularly the lung--as "inflammatory pseudotumors" or "plasma cell granulomas." In our opinion, these lesions also bore a superficial resemblance to cutaneous lymphoid hyperplasia secondary to insect bites. Other differential diagnostic considerations include dermatofibroma, nodular fasciitis, Kimura's disease, epithelioid (histiocytoid) hemangioma, and reactive lymph nodes.

Adult

Dermal thymus. A light microscopic and immunohistochemical study.

Two rare cases of distinctive thymic remnants occurring in the skin are described. The lesions were present at birth, and involved the side of the neck. One child had a complete cleft lip and palate. The other had multiple congenital anomalies consistent with a rare syndrome entitled branchio-oculo-facial syndrome. Microscopically, lobular foci were present in the dermis and consistent with both prethymic and thymic remnants. Immunohistochemical studies using antibodies to T cells and B cells showed a distribution similar to that seen in normal thymus. Dermal thymus appears to be a distinct entity and may be associated with other faciobranchial defects.

Abnormalities, Multiple