Eccrine syringofibroadenoma surrounding a squamous cell carcinoma: a case report.

A 91-year-old man presented with a 9.0 x 7.0 cm exophytic mass on the dorsum of the right foot, surrounded by a scaling hyperkeratotic plaque-like lesion that had been present for many years. He had similar long-standing hyperkeratotic plaque-like lesions on both legs. Histopathologic examination of the exophytic mass revealed a well-differentiated squamous cell carcinoma surrounded by an eccrine syringofibroadenoma (ESFA). Histochemistry, immunohistochemistry and electron microscopy support this diagnosis. To our knowledge, this is the only reported case of ESFA being intimately associated with a malignant neoplasm.

Penectomy in diabetic patients undergoing maintenance dialysis.

Penectomy was performed to sustain life in 2 patients with insulin-dependent and non-insulin-dependent diabetes mellitus, respectively, who were undergoing maintenance hemodialysis. Both patients previously had manifested a series of serious macro- and microvascular diabetic complications. The histopathologic findings in both cases included gangrenous necrosis of penile tissue, while case 2 also evinced calcification of penile arteries. Penectomy has been reported as the result of penile malignancy, anticoagulant toxicity, self-inflicted injury, and criminal assault. Other reports document penectomies attributed to perineal infection (Fournier's syndrome) in diabetic patients with uremia. In five previously reported cases of penectomy in diabetic patients undergoing dialysis, systemwide arteriopathy was present in all. There is an association between uremia in diabetics and predisposition to an ischemic-infectious lesion of the penis that fails to respond to antimicrobial therapy.

Foreign-body osteitis of the metacarpal bone.

Foreign-body granulomas in the hand can appear secondary to an unrecognized foreign material. In a six-year-old boy, an extensive sterile osteitis of the second metacarpal bone appeared adjacent to a wood splinter.

Burkitt's lymphoma in a patient with systemic lupus erythematosus.

Patients with systemic lupus erythematosus (SLE) appear to be at increased risk for development of neoplastic disease. We describe the case of a male teenager with SLE and Burkitt's lymphoma. His presentation was similar to that of an exacerbation of his underlying SLE. We believe this to be the first case of Burkitt's lymphoma in a patient with SLE. The association of SLE and malignancy, with emphasis on lymphoproliferative states, is discussed.

Perinatal nonimmune hydrops: diagnostic ultrasonography and related aspects of management.

Nonimmune hydrops fetalis may become the commonest form of hydrops seen in Western countries during the perinatal period, and it has at least a 50% mortality. This report describes five infants with nonimmune hydrops associated with maternal hydramnios and with congenital fetal lesions or disorders, ie, mediastinal teratoma, pulmonary leiomyosarcoma, Beckwith-Weidemann syndrome with omphalocele, fetal tachycardia, and Down's syndrome. Three of the infants survived the neonatal period and two of these underwent surgery for resection of their tumors early in the neonatal period. The third had an omphalocele repaired at 6 hours of age. The literature is reviewed with respect to the pathophysiology of nonimmune hydrops. Its diagnosis and treatment are discussed, with special emphasis on the role of ultrasound in its early diagnosis and optimal prenatal and postnatal management, and on the morbidity seen in survivors.

Congenital oral cyst with heterotopic gastrointestinal and respiratory mucosa.

A congenital oral duplication cyst of the ventral surface of the tongue was found to contain both gastrointestinal and respiratory epithelium. The literature is reviewed, with emphasis on the possible histogenesis of this extremely rare lesion, as well on the clinical differential diagnosis.

Cystosarcoma phylloides in the adolescent female.

Between 1979 and 1985, five adolescent females have undergone excision of cystosarcoma phylloides. Mean age at presentation was 15 years with a range of 13 to 18 years. Clinical characteristics unique to this diagnosis included a recent history of rapid growth and the large size of the breast mass (mean diameter 7 cm) at initial presentation. Each patient underwent "cosmetic" excisional biopsy utilizing either a circumareolar or inframammary approach. In four patients, the neoplasm was benign with no postoperative recurrence (mean follow-up 33.8 months). The remaining lesion was malignant (liposarcoma) and subsequent simple mastectomy was performed. There was no evidence of disease at 35 months.

Chromosome abnormalities in familial hemophagocytic lymphohistiocytosis.

Familial hemophagocytic lymphohistiocytosis (FHLH) is an uncommon disorder characterized by multiorgan infiltration with phagocytic histiocytes/macrophages. It may be inherited as an autosomal recessive trait, but specific associated cytogenetic abnormalities have not been documented. The authors describe a 10-week-old white female without prior family history of FHLH, who fulfilled the histologic and clinical criteria for the diagnosis. In addition, cytogenetic abnormalities, including the presence of double minute chromosomes and occasional loss of chromosomes 7 and 12, were documented in unstimulated peripheral blood cells. These karyotypic findings are usually associated with dyserythropoietic and leukemic states, and have not been described previously in the context of FHLH. It may be useful to do chromosome analyses on unstimulated peripheral blood cultures from FHLH patients before treatment to examine the karyotype of proliferating cells, which may represent the infiltrative histiocytes seen in the disorder.

Liposarcoma arising within a cystosarcoma phyllodes.

A liposarcoma arising within a cystosarcoma phyllodes in a 17-year-old black female was treated by total mastectomy without the need of radiation and/or chemotherapy. The patient is alive and well with no evidence of recurrence or metastasis at 20 months recent follow-up. The clinicopathological features of malignant cystosarcoma phyllodes-liposarcoma are discussed.

Primary bronchopulmonary leiomyosarcoma in childhood.

There have been only nine cases of primary bronchopulmonary leiomyosarcoma (PBPL) in children reported in the English language literature, to our knowledge. Herein, we report the tenth case of PBPL in a 24-hour-old newborn infant with associated nonimmunologic hydrops fetalis. We believe this to be the youngest patient ever reported to have PBPL, and the first such tumor, to our knowledge, to be a possible cause of nonimmunologic hydrops fetalis. The patient was alive and well 34 months after a right-sided lower lobectomy, without the need for radiotherapy and/or chemotherapy. We review the clinical pathologic features, treatment, and follow-up of PBPL.

Canine rhinosporidiosis in Arkansas.

Rhinosporidiosis is a fungal infection caused by Rhinosporidium seeberi, a microorganism of complicated, undetermined life cycle. Many species of animals are susceptible to rhinosporidiosis, including human beings. The frequency of occurrence of the disease in domestic animals is unknown. A one-year-old male Collie dog from Northeast Arkansas was found to have rhinosporidiosis presenting as an intranasal polypoid mass. We believe this is the first documented report of rhinosporidiosis from the State of Arkansas.

Primary intracranial immunoblastic lymphoma in infancy.

Primary intracranial lymphoma is uncommon in any age group, but it is especially rare in childhood. This report describes a previously healthy, 14-month-old female infant who developed a primary intracranial immunoblastic (probable B-cell) lymphoma which remained confined to the central nervous system until the time of death, 23 months after diagnosis. She appears to be the youngest patient with documentation of such a diagnosis by light and electron microscopy and by histochemical and immunoperoxidase studies. An immunological investigation was negative. Significant maternal and paternal family histories of malignancy suggest that a genetic predisposition, combined with postzygotic events such as viral infection, may be responsible for this familial cluster of tumors, and for this patient's unusual presentation.

Acute cytoreduction techniques in the early treatment of hyperleukocytosis associated with childhood hematologic malignancies.

Early and effective cytoreduction for high peripheral white blood cell counts in pediatric patients with acute leukemia may be helpful in preventing complications secondary to hyperviscosity. It also may be a useful adjunct to systemic chemotherapy. As an alternative to automated apheresis for this purpose, manual exchange transfusion is efficacious and does not require hemapheresis instrumentation and disposables and the related special staff. Two patients, a neonate with acute myeloblastic leukemia and a white blood cell count of 422.2 k/microliter as well as a 2 1/2-year-old with an admission diagnosis of acute promyelocytic leukemia and a white blood cell count of 617.4 k/microliter, underwent manual exchange hemotherapy for acute cytoreduction. The procedures were tolerated well, and significant leukocyte removal was achieved, with the respective leukocyte reductions being 81.1 and 68.7%. The techniques available for pediatric cytoreduction are compared, with emphasis on their efficiency and safety and appropriateness for very small children.

Anaphylactic transfusion reaction associated with a possible anti-A2m(1).

A 42-year-old leukaemic black male developed shortness of breath, itching, hot flushes, substernal pain, marked hypotension, and cardiorespiratory arrest after the infusion of 50 ml of pooled platelet concentrates. The patient had had one previous and uneventful red blood cell transfusion. Serologic studies of this patient's post-transfusion serum showed the presence of an antibody against one denatured IgA2 protein with the phenotype A2m(1 + 2-). All of the platelet donors were A2m(1) positive. The patient was successfully transfused with platelets from donors with the phenotype A2m(1 - 2+). Although 35.8% of Blacks and 30.0% of Chinese are A2m(1) negative, this is the first reported transfusion reaction associated solely with the occurrence of a possible anti-A2m(1) in an individual of the type A2m(1 - 2+).

ACTH-producing pheochromocytoma.

A benign adrenal medullary tumor that secreted adrenocorticotropic hormone (ACTH) was associated with bilateral adrenocortical hyperplasia and clinically evident Cushing syndrome. The clinical and chemical features were those usually associated with pituitary Cushing disease, including partial suppression of urinary OH steroids after administration of 8 mg of dexamethasone. The fractionization of the tumor's ACTH revealed 70% little "biologically active" ACTH, which is usually found in this concentration only in pituitary tissue.