Subcutaneous fat necrosis in a newborn following icebag application for treatment of supraventricular tachycardia.

Cases of subcutaneous fat necrosis of the newborn (SCFN) and neonatal cold panniculitis have been reported most often secondary to perinatal distress or hypothermia. We present a case of a newborn infant who developed erythematous, indurated plaques on both cheeks and right shoulder following ice pack application for supraventricular tachycardia. The distinction between SCFN and cold panniculitis is important as SCFN may have complications such as hypercalcemia, whereas cold panniculitis is not associated with such sequelae. Clinicians should be aware of the diagnostic similarities and differences between these two conditions because of differences in potential serious sequelae.

Noninvasive papillary serous carcinoma of the endometrium.

OBJECTIVE: To determine the clinical course of noninvasive uterine papillary serous carcinoma and whether it indicates advanced metastatic disease. METHODS: We reviewed the charts of women with noninvasive uterine papillary serous carcinoma who were treated at our institution and abstracted surgical stage, sites of metastases, disease progression, and length of follow-up. RESULTS: There were 595 cases of endometrial adenocarcinoma between January 1990 and February 2000, 69 of which had papillary serous histology. Sixteen were noninvasive tumors. Six were confirmed stage IA by complete surgical staging and ten were associated with metastasis at staging. Two of the six women with stage IA tumors had disease recurrence. CONCLUSIONS: Noninvasive papillary serous carcinoma is often widely metastatic. In our experience, approximately two thirds of patients had metastasis, indicating the need for complete surgical staging. Even in those with disease limited to the endometrium, a significant percentage will have disease recurrence.

Immunohistochemical evidence for the vascular origin of primary adrenal pseudocysts.

Pseudocysts are the most common nonfunctioning cystic adrenal lesions associated with symptoms. We have studied tissue from two patients with large adrenal pseudocysts in whom no antecedent cause could be documented. Immunohistochemical examination of these formaldehyde-fixed paraffin-embedded tissues with antibodies directed against the major proteins of the basement membrane (laminin and type IV collagen) revealed intense linear staining surrounding the cystic spaces and at the compressed adrenal cortical/pseudocyst interface. These findings lead us to conclude that these lesions are vascular in nature and, therefore, closely related to the previously recognized adrenal cysts of endothelial origin.

The pathologic spectrum of small cell carcinoma of the cervix.

Small cell carcinoma of the cervix is a term used to describe several entities including cervical carcinoid, "oat cell" carcinoma, reserve cell carcinoma, and poorly differentiated nonkeratinizing squamous cell carcinoma. The light microscopic, ultrastructural, and clinical features of seven small cell cervical carcinomas are presented in this report. Five tumors in our report were diagnosed as small cell anaplastic or oat cell carcinoma by light microscopy. This diagnosis was associated with a poor prognosis, and four of these patients were dead of disease within 24 months. The ultrastructural features of these tumors were similar, but neurosecretory granules were demonstrable in only three of the five cases. Evidence of glandular differentiation was present in one of the five cases, and dual differentiation, i.e., neuroendocrine and squamous, was noted in another. The similarities between pulmonary small cell carcinoma and these cervical lesions are discussed and the literature is reviewed. Also included in the study is a well-differentiated neuroendocrine lesion, a carcinoid that behaved in an aggressive fashion. The final case was a carcinoma putatively derived from reserve cells. The features that help make the diagnosis of reserve cell carcinoma and that distinguish this lesion from other small cell carcinomas are presented.

Epithelioid blue nevus: neoplasm Sui generis or variation on a theme?

The epithelioid blue nevus has recently been associated with the Carney complex, which is characterized by myxomas, spotty skin pigmentation, endocrine overactivity, and schwannomas. Using the general criteria proposed by Carney and Ferreiro, similar lesions were identified in 33 patients with no evidence of the Carney complex. Those lesions presented on the face, trunk and extremities of 15 males and 18 females. The mean age was 35 years, much older than those in the Carney complex (mean 16.3 years). Clinical diagnoses included malignant blue nevus, atypical nevus, melanoma, congenital nevus, and dermatofibroma. The lesions were symmetric, predominantly dermal melanocytic proliferations arranged as short fascicles, small nests, and single cells. Large polygonal and epithelioid melanocytes with moderate pleomorphism, and occasional nuclear pseudoinclusions were admixed with heavily pigmented dendritic and spindled melanocytes and melanophages. Rare mitotic figures were seen in some cases. The neoplasms showed a morphologic spectrum that encompassed a group of combined blue nevi with epithelioid melanocytes and other Spitz's nevus characteristics. These epithelioid combined nevi (ECN) fell into three phenotypes with morphologies that most closely paralleled those pictured by Carney and Ferreiro in the Carney complex: the classic or Carney complex pattern (ECN-CC), those that showed overlap with deep penetrating nevus (ECN-DPN), and those that have many dermal Spitz's nevus features, [BLue + SpITZ's nevus; (ECN-BLITZ)I. In six cases, there was such an admixture of features that it was difficult to ascribe them to one of the groups. Nine lesions had associated banal congenital nevus. Follow-up that averaged over 2.5 years (31 months) (range 6-162 months) showed no evidence of malignancy or recurrent disease after excision. Epithelioid combined nevus is a type of combined nevus with blue nevus and Spitz's nevus features, which may or may not be associated with the Carney complex. It shows morphologic overlap with the epithelioid blue nevus described by Carney (ECN-CC), deep penetrating nevus (ECN-DPN), and blue nevus with intradermal Spitz's (desmoplastic) nevus (ECN-BLITZ). Epithelioid combined nevus is thought to be a fitting nosologic designation for all of these lesions.

Panniculitis in juvenile dermatomyositis.

Panniculitis is a rarely reported clinical finding in dermatomyositis. We present a 14-year-old African American boy with a 4-year history of dermatomyositis referred for evaluation of tender, indurated plaques and nodules on the trunk and proximal extremities. A biopsy specimen revealed epidermal and dermal changes consistent with dermatomyositis. Although calcification was absent, a striking lobular panniculitis was observed. A total of seven cases of clinical panniculitis in association with dermatomyositis have been published. As in our patient, it typically presents as indurated, tender plaques and nodules on the arms, thighs, and buttocks. Although in the vast majority of cases panniculitis found in association with dermatomyositis is subclinical and represents an incidental histopathologic finding, the case we present further emphasizes the need to recognize panniculitis as a rare, but clinically relevant pathologic feature seen in certain lesions of juvenile dermatomyositis.

Apocrine poroma: a distinctive case in a patient with nevoid basal cell carcinoma syndrome.

Traditionally, poromas have been classified as eccrine neoplasms, but several recent reports of poroid tumors with sebaceous, follicular, and apocrine differentiation have challenged this idea. In support of alternative differentiation, a case of an "apocrine" poroma is reported in a 19-year-old man with the nevoid basal cell carcinoma syndrome. A papule on the right cheek, thought clinically to be a basal cell carcinoma, was excised. Anastomosing lobules of small uniform basaloid (poroid) cells formed small ductular structures lined by eosinophilic cuticles and extended into the superficial reticular dermis. The neoplasm originated from follicular infundibula and was surrounded by a myxoid stroma. Focally, primitive hair bulb and papillae differentiation was present, and some of the ducts were lined by cells suggesting decapitation secretion. The histologic pattern and the common embryologic origin of the folliculosebaceous-apocrine unit support apocrine differentiation of this tumor. The association with the nevoid basal carcinoma syndrome appears to be unique. This case, in addition, demonstrates overlapping features with the infundibulocystic type of basal cell carcinoma commonly seen in the basal cell nevus syndrome.

CD34-positive eruptive fibromas.

The list of entities comprising a proliferation of CD34 (+) spindle cells continues to grow. Described, herein, is a patient who had an indolent eruption of scattered papules composed of CD34 (+) spindle cells, beginning in adolescence. An 18-year-old female patient presented with asymptomatic, tan/brown papules over the neck, chest, and proximal extremities. They appeared 6 years previously and had slowly increased in number. Biopsy from the neck showed a proliferation of plump spindle cells, associated with delicate collagen, in the upper reticular dermis. No atypia nor mitotic figures were present. The spindle cells were negative for S-100, muscle actins, and Factor XIIIa, but stained intensely with CD34. This unusual mesenchymal proliferation of CD34 (+) apparent dermal dendrocytes did not have the storiform pattern, short fascicles, nor mitotic figures of DFSP. The completely negative muscle markers helped to exclude dermatomyofibroma, and no morphological evidence of vasoformative differentiation was seen. The clinical picture militated against solitary fibrous tumor. These eruptive tumors are benign and thought to represent a distinctive fibroma produced by proliferated CD34 (+) stromal cells.

Primary transitional cell carcinoma of the female urethra with features of clear cell adenocarcinoma.

Primary malignant urethral neoplasms are unusual in women. Those of mixed histologic type are so rare as to define adequate documentation. We report the case of an elderly black woman with an urethral carcinoma which had features of a transitional cell carcinoma as well as clear cell adenocarcinoma. The histologic and ultrastructural features are presented and the histogenesis is discussed. Although the patient had both high grade and high stage disease and failed initially after external beam radiation therapy, she is alive and well, without evidence of recurrent disease, 19 months after anterior exenteration.

Autosomal dominantly inherited generalized basaloid follicular hamartoma syndrome: report of a new disease in a North Carolina family.

BACKGROUND: An 8-year-old girl presented with hundreds of milia, measuring 1 to 2 mm; comedone-like lesions; skin-colored and hyperpigmented papules on the face, scalp, ears, neck, upper trunk, and lower arms along with diffuse scalp hypotrichosis; and pinpoint palm/sole pits. Onset was in early childhood and the disease was historically present in 6 generations. OBJECTIVE: Our objectives were to delineate the clinical and histopathologic features and mode of inheritance as a base for gene studies. METHODS: Eighteen family subjects were studied. Twenty-six skin biopsy specimens were examined. A detailed pedigree was constructed. A complete literature search was done concerning diseases with generalized basaloid follicular hamartomas. RESULTS: The lesions were basaloid follicular hamartomas and other folliculocentric abnormalities. Inheritance was autosomal dominant. Extensive literature search confirmed the finding of a unique genodermatosis. CONCLUSION: A new genodermatosis termed dominantly inherited generalized basaloid follicular hamartoma syndrome was defined by delineating its clinical and histopathologic features and mode of inheritance and by extensive literature review.

Expression of Ki-67 in vulvar carcinoma and vulvar intraepithelial neoplasia III: correlation with clinical prognostic factors.

OBJECTIVES: In vulvar carcinoma, the expression of Ki-67 has been previously found to correlate with patient outcome. The objective of the study was to determine whether a specific pattern of expression was associated with occult vulvar cancer in patients with vulvar intraepithelial neoplasia (VIN) III and whether patterns of Ki-67 expression correlated with other clinical prognostic factors. METHODS: 19 women with only VIN III, 16 women with both vulvar cancer and VIN III, and 15 women with only vulvar cancer were identified. Immunostaining, using a monoclonal antibody for Ki-67, was then performed on representative tissue blocks and slides were assessed for diffuse or localized patterns of expression. For the patients with vulvar cancer, the type of staining was correlated with FIGO stage, tumor grade, lymph nodes status, and associated VIN III. RESULTS: All 35 patients with VIN III exhibited a diffuse staining pattern. In the 31 patients with vulvar carcinoma, 11 (35%) expressed a diffuse staining pattern while 20 (65%) showed a localized pattern. Poorly differentiated tumors were associated with a diffuse staining pattern (P = 0.013, RR 3.59, CI 1.59-7.60). For vulvar carcinoma, there were no statistically significant relationships between Ki-67 expression pattern and stage, associated VIN III, or lymph node involvement. CONCLUSION: VIN III, regardless of a concomitant vulvar cancer, always expressed a diffuse pattern; thus Ki-67 staining was not useful as a marker for occult cancer. In women with vulvar carcinoma, however, a diffuse Ki-67 expression was significantly associated with poorly differentiated tumors.

Appendix cancer mimicking ovarian cancer.

Appendiceal adenocarcinoma is a rare malignancy for which there is no characteristic clinical presentation. We describe five women who presented with signs and symptoms characteristic of advanced ovarian cancer but whose final diagnosis was stage IV appendiceal cancer. Between 1998 and 1999, five women treated for presumed ovarian cancer were identified as having primary appendiceal cancer. Medical records and pathology were retrospectively reviewed. The median age was 47 years (range 36-61 years). All had elevated preoperative CA125 levels with a median value of 171 micro/ml (range 46-383). Four women underwent right hemicolectomy with two requiring radical surgical tumor debulking to render them optimally debulked. Four had postoperative chemotherapy, the most common agent used was 5-flourouracil. Median survival was 6.75 months (range 19 days-11 months). Primary adenocarcinoma of the appendix is rare; therefore, the clinical utility of radical tumor debulking and chemotherapy is not well described. Given the poor survival in our series, all efforts should be considered palliative. Although this disease process is uncommon, it should be entertained by gynecologic oncologists in the differential diagnosis of an intra-abdominal mass and ascites. The ability to make the correct diagnosis and differentiate between an ovarian and appendiceal primary is critical as the treatment modalities vary.

Esophagitis: a frequent consequence of gastroesophageal reflux in infancy.

A control group of infants was evaluated to determine criteria for the diagnosis of histologic esophagitis. Based on our observations, histologic esophagitis was defined as four or more intraepithelial neutrophils or one eosinophil per high power field or both. Esophageal biopsy specimens from 33 consecutive infants younger than 2 years who had been examined for clinically significant gastroesophageal reflux (GER) were reviewed for histologic esophagitis. Endoscopy had been performed in each patient, and 4.1 +/- 1.1 (mean +/- SD) biopsy specimens had been obtained above the distal 20% of the esophagus. Twenty (61%) infants had histologic esophagitis, including 15 with intraepithelial eosinophils alone, one with intraepithelial neutrophils alone, and four with both. Older infants (7 to 24 months) with histologic esophagitis were more likely to have moderate to severe inflammation than were infants younger than 7 months of age (P = 0.01). Endoscopic evidence for gross esophagitis was found in six (18%) infants; of these, five had abnormal biopsies, including four with moderate to severe inflammation. Among the 27 infants with a grossly normal esophagus, 14 (52%) had histologic esophagitis, including nine (33%) with moderate to severe inflammation. We conclude that in infants with clinically significant GER: (1) esophagitis is common, (2) histologic esophagitis frequently occurs in the absence of gross endoscopic findings, (3) the likelihood of moderate to severe inflammation increases after 6 months of age, and (4) intraepithelial eosinophils are a sensitive marker for acute inflammation in association with GER.