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Biomedical subjects

R L Sánchez

Publications and source records attributed to R L Sánchez.

11 recordsLinked to original sources

Atrophic and plaquelike dermatofibrosarcoma protuberans.

It is clinically important to realize that dermatofibrosarcoma protuberans (DFSP) often begins as a nonprotuberant plaque and occasionally persists as such. Cases of what has been called atrophic DFSP have recently been reported. None of these cases varied either histologically or prognostically from classical nodular DFSP, and all should be considered part of the clinical spectrum of DFSP. However, histologic variants of DFSP can portend a worse prognosis. The fibrosarcomatous variant, which is fascicular and does not immunostain with anti-CD34, is an example. Recently, another fascicular variant that does positively immunostain with anti-CD34 has been described as plaquelike DFSP. We described a case of a DFSP that had histologically banal, slender fascicles of anti-CD34 immunostaining spindle cells in a clinically nonprotuberant plaque despite subsequently developing a nodule with typical storiform histology. The importance of anti-CD34 immunoperoxidase staining in the diagnosis and prognosis of DFSP variants is emphasized.

Antigens, CD34↗

Multiple angiolymphoid hyperplasia with eosinophilia of the hand: report of a case and review of the literature.

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare angioproliferative disorder with a predilection for the head and neck region. ALHE is very rare in the extremities and even more rare in the hand, with only single cases reported in this location. We describe the occurrence of multiple lesions of ALHE on the right hand of a 26-year-old woman. We report this unusual occurrence and convey a clinical differential diagnosis with other benign and malignant conditions of the hand. All the lesions of the palm were excised out as a unit, the subungual lesion removed completely, and the involved segment of the right common digital artery excised. The tissues were processed for routine histologic examination as well as for histochemical and immunohistochemical tests. Electron microscopic study was also performed. Recurrent lesions on the same hand were also excised. Histologic examination of all the lesions revealed classic morphologic features of ALHE. The results of special studies confirmed the endothelial nature of the proliferating cells. ALHE can present as multiple lesions in the hand, and can arise in a subungual location. The possibility of larger arteries being involved should be considered. Recurrent lesions may appear months after the original presentation.

Adult↗

Multiple cutaneous myxomas. Report of a case without other elements of Carney's complex.

An 11-year-old hispanic female was referred for evaluation of multiple asymptomatic papules which had slowly developed since age two. The patient had no significant medical history and no family members, including an older sibling, were similarly affected. Physical examination revealed multiple 2-6 mm flesh-colored to erythematous papules over the posterior scalp, trunk, and extremities, predominantly in a midline distribution. No lentigines, blue nevi, or evidence of endocrine ab-normalities were noted. Echocardiogram was normal with no evidence of myxoma. Six cutaneous biopsies showed myxomas, as evidenced by well-circumscribed dermal tumors composed of stellate, dendritic cells embedded in a mucinous stroma. Special stains and electron microscopy confirmed the H&E finding. Immunoperoxidase staining for Factor XIIIa was positive in the dendritic tumor cells, suggesting a relationship with dermal dendrocytes. This patient represents a case of multiple cutaneous myxomas without other components of Carney's complex of myxomas, spotty pigmentation, endocrine overactivity, and a variety of uncommon tumors. A long-term follow-up is indicated since some of the above cited elements of the syndrome could appear over time.

Back↗

AgNOR area measurements differentiate benign and malignant melanocytic lesions more accurately than simple counting.

Comparison of argyrophilic nucleolar organizer region (AgNOR) counts has been found to yield statistically significant differences between benign and malignant conditions albeit with considerable overlap. In this study we compared the size of AgNORs and the nuclear areas, as well as the AgNOR count in 23 benign melanocytic lesions and 9 melanomas. Of these parameters, the ratio AgNOR area/nuclear area was found to be the main discriminating factor between melanoma and all the other benign groups studied, with p values of < 0.01 and no overlap. Next to it was the nuclear area, which in our study gave significant differences between the groups evaluated. The total AgNOR area and the largest AgNOR gave results comparable to the simple AgNOR counting in all groups studied, except in Spitz nevus, in which the AgNOR counts were less significant than the other parameters (p = 0.0420). We conclude that the ratio AgNOR area/nuclear area discriminates benign from malignant melanocytic lesions better than AgNOR counts or other parameters studied.

Cell Nucleus↗

Clinical and histologic features of striated muscle hamartoma: possible relationship to Delleman's syndrome.

We describe 5 new cases of striated muscle hamartoma (SMH) and review the 7 cases previously described in the literature. Eleven of the 12 patients had single lesions centrally located on the chin (4), near the ala of the nose (3), on the upper chest (3), and on the upper lip (1). Histologically all of the lesions were characterized by a central core containing bundles and individual fibers of skeletal muscle as well as other mesenchymal elements. The muscle fibers penetrated the dermis among numerous folliculo-sebaceous structures. Of the 12 cases of SMH, 3 had other associated congenital anomalies including a cleft lip in 1 patient and a thyroglossal duct sinus in another with single lesions, and preauricular sinuses, low-set ears and bilateral sclerocorneas in the case of multiple SMH. The relationship of SMH with oculocerebrocutaneous syndrome is discussed, and we conclude that at least some of the skin appendages associated with that syndrome are SMH.

Abnormalities, Multiple↗

Dermatomyofibroma: report of two cases.

Dermatomyofibroma is a recently described, benign acquired cutaneous proliferation of fibroblasts and myofibroblasts. We report 2 additional cases, both in young women. The lesions measured 0.5 and 1.0 cm and were located on the breast and upper back. Follow-up at 8 months and 3 years revealed no evidence of recurrence. Both lesions exhibited a uniform population of spindle cells forming intersecting long fascicles with a tendency to horizontal orientation in the reticular dermis. Cytologic atypia was absent and mitotic figures were minimal or absent. Collagen was present as thin fibers separating individual cells and as thicker bundles between the fascicles. A fine reticulin network surrounded the spindle cells. Elastic fibers were mostly preserved and appeared thicker or even increased in number. The spindle cells were immunoreactive for vimentin and muscle actin and negative for desmin, S-100 protein, factor XIIIa, and CD34. Electron microscopic studies confirmed the presence of myofibroblasts. The differential diagnosis was made with cellular and fibrous dermatofibroma, piloleiomyoma, hypertrophic scar, and dermatofibrosarcoma protuberans, both classic and plaque type. The data presented supports the histologic distinction of dermatomyofibroma from other fibroblastic proliferations of the skin.

Adult↗

Prostatic acid phosphatase in strumal carcinoids of the ovary. An immunohistochemical study.

BACKGROUND: Strumal carcinoids (SC) are ovarian tumors containing thyroid parenchyma admixed with carcinoid elements. Microscopically, the carcinoid component of SC usually presents a ribbon or trabecular pattern similar to the pattern exhibited by hindgut carcinoids. The authors designed an immunohistochemical study to ascertain further similarities of the carcinoid component of SC to rectal carcinoids. METHODS: Five cases of SC were examined by light microscopic study with hematoxylin and eosin and immunoperoxidase staining with a group of neurohormonal peptides, thyroglobulin, prostatic acid phosphatase, and prostate specific antigen. RESULTS: All tumors were positive for thyroglobulin, chromogranin, prostatic acid phosphatase (PAP), and glucagon. They were consistently negative for prostate specific antigen and variably positive to the other antibodies. CONCLUSIONS: The consistent positivity of the carcinoid component of SC to PAP immunostaining is a further similarity of these tumors to rectal carcinoids, in which positive immunostaining to PAP has been observed. The similarity is not well understood, however, as all evidence points to the origin of SC from germ cells in mature cystic teratomas without any embryologic relationship to the hindgut.

Acid Phosphatase↗

Dendrocyte population in cutaneous and extracutaneous Kaposi's sarcoma.

In order to investigate the hypothesis that the spindle cells of Kaposi's sarcoma originate from dendrocytes, we stained 22 cases of cutaneous and extracutaneous Kaposi's sarcoma for the presence of factor XIIIa. We selected eight lesions from the skin, five from the oral mucosa, four from gastrointestinal mucosa, three from lymph nodes, and one each from esophagus and conjunctiva for this study. The majority of cutaneous and lymph node Kaposi's sarcoma lesions had a considerable number of dendrocytes admixed with the spindle tumor cells. Of the five oral mucosal lesions only three showed a focal presence of dendrocytes. No dendrocytes were observed in the lesions biopsied from the gastrointestinal mucosa, esophagus, and conjunctiva. Our findings suggest that the proliferation of dendrocytes seen within the lesions of Kaposi's sarcoma in the skin and lymph nodes probably represents a nonspecific host response in organs where they are normally present, and it is unlikely that they constitute the neoplastic cells of Kaposi's sarcoma.

Dendritic Cells↗

Dysplastic nevi of the scalp and forehead in children.

To determine if there is a significant difference in the relative frequency and degree of atypia of sporadic dysplastic nevi from the scalp, face, and neck area in children as compared with nevi from the rest of the body, we reviewed 99 consecutive biopsy specimens of melanocytic nevi from the scalp, face, and neck areas in children less than 18 years of age and compared them with 95 consecutive cases of nevi from other areas of the body in children of the same age. Large numbers of the nevi biopsied from the scalp (13 of 31; 41.93%) and forehead (2 of 10; 20%) were dysplastic. The number of dysplastic nevi from the neck (1 of 58; 1.72%) was not assessed as very different from the incidence found in other regions of the body, where 7 dysplastic nevi (7.36%) from a total of 95 nevi were found. Of the 13 dysplastic nevi from the scalp, 9 showed minimal atypia and 4 showed moderate atypia. No nevi with severe atypia were found. Many pigmented nevi from the scalp and forehead in children in this study were dysplastic. This finding points out the importance of examining the scalp of children for the presence of dysplastic nevi. The majority of nevi from the neck were common nevi.

Adolescent↗

Nonlipidized juvenile xanthogranuloma: a histologic and immunohistochemical study.

Among all patients with a pathologic diagnosis of juvenile xanthogranuloma (JXG) seen at our institution from 1983 to 1994, we identified five patients with an unusual histologic pattern that differed from the classic juvenile xanthogranuloma (CJXG) with foamy cells and Touton giant cells. Four of these five cases, which we termed nonlipidized juvenile xanthogranuloma (NJXG), were seen in infants. The histologic features include a monomorphic infiltrate with absent or few foam cells and Touton giant cells. There is little inflammation, and mitotic figures are easily found. Four cases exhibit a diffuse sheetlike pattern while one is trabecular. Immunoperoxidase staining was done. All lesions are consistently positive for factor XIIIa as opposed to only focally positive or negative in CJXG and negative in Langerhans cell histiocytosis (LCH). The S-100 was negative. NJXG represents an atypical histologic variant of JXG, which may suggest a malignant or aggressive tumor. The follow-up, however, indicates that these lesions behave in a fashion similar to those of CJXG. The differential diagnosis should be made with LCH, intradermal nevus, and reticulohistiocytosis. The immunoperoxidase findings help to differentiate NJXG from these entities.

Child, Preschool↗