Steatocystoma multiplex with bilateral preauricular sinuses in four generations.

A rare case of steatocystoma multiplex and bilateral preauricular sinuses in four generations is presented along with a review of the literature. The cause, pathogenesis, and treatment of steatocystoma multiplex is discussed. Steatocystoma multiplex (SCM) is a rare clinical disorder characterized by numerous recurrent cutaneous cysts. Since the first case described by Bosellini in 1898, both nonfamilial and autosomal dominant inheritance patterns have been described. There have been at least 13 well-documented nonfamilial cases. However, since Noonjin and Reynolds in 1948 first described the autosomal dominant inheritance pattern, only 4 well-documented familial cases have been reported. To date, there has been one case report of SCM in four generations, and that case was not associated with any other anomaly. The present case describes the occurrence of SCM in association with bilateral preauricular sinuses in multiple generations.

Obstructing fallopian tube papilloma.

A grossly well-defined papilloma was found in the fallopian tube of a 41-year-old woman who had a total hysterectomy and bilateral salpingo-oophorectomy for carcinoma in situ of the cervix. The papilloma was unassociated with significant inflammation, hormone administration, or endogenous hormone excess. It presented as a hydrosalpinx developing over a period of several months. This may represent the second true fallopian tube papilloma to be recorded.

Ectopic twin gestation discordant for anencephaly. A case report.

A 30-year-old white woman thought to have a 12-week intrauterine pregnancy developed a large cul-de-sac mass, which proved to be an ectopic, monoamnionic twin pregnancy (males) discordant for anencephaly. While twin pregnancies discordant for neural tube defects are well known, the extrauterine location made this case remarkable.

Acute febrile neutrophilic dermatosis (Sweet's syndrome) in a patient with hairy-cell leukemia.

A case of hairy-cell leukemia that started with cutaneous lesions similar to those of acute febrile neutrophilic dermatosis (Sweet's syndrome) is reported. The patient had leukopenia and a recurrent eruption for a year prior to the diagnosis of the hematologic disorder. Bone marrow examination eventually demonstrated characteristic "hairy" cells with tartrate-resistant, acid-phosphatase activity. Biopsy of a cutaneous lesion suggested an abscess showing a dense neutrophilic dermal infiltrate with perivascular predilection. The findings of IgA, IgM, C3, and fibrinogen in vessel walls by immunofluorescence and vascular disruption by electron microscopy contribute additional evidence for an immunologic determinant in this instance of Sweet's syndrome. It is known that neutrophilic infiltrates of Sweet's syndrome may have a variety of clinical presentations. This is the second case of hairy-cell leukemia with which Sweet's syndrome was associated.

Papovavirus and squamous cell carcinoma.

There is considerable interest in the role of the human wart virus in the pathogenesis of squamous cell carcinoma. Mucosal wart virus infections may produce considerable epithelial atypia and have eventuated in carcinoma in "normal" individuals in some instances. The verruciform lesions of patients with epidermodysplasia verruciformis frequently have this outcome. The finding of a virus of papova morphology in the noncondylomatous epithelium adjacent to a squamous cell carcinoma of the tongue in a young immunosuppressed patient is described. Such a finding adds credibility to the belief that a virus of the papova group may be integral to neoplastic evolution in at least some instances.

Primary adenocarcinoma of the fallopian tube. A case report with discussion of its pattern of endometrial spread and possible resulting diagnostic confusion.

The case of a 59-year-old woman with primary adenocarcinoma of the fallopian tube is reported. The case presented with pain and a pelvic mass, and at laparotomy appeared to represent severe pelvic inflammatory disease with a hydrosalpinx. Histologically a poorly differentiated adenocarcinoma of the fallopian tube was found. There was contiguous spread of the tumor to the endometrial surface, to endometrial polyps, and to the opposite fallopian tube. The difficulty of clinical diagnosis is discussed, especially in regard to the pattern of endometrial spread.

Pleomorphic pulmonary hamartoma: an apparently unique variant of pulmonary hamartoma.

Pulmonary hamartomas of limited variety have been described. Most present as asymptomatic coin lesions in adults and consist of mesenchymal tissue, usually cartilage, in combination with irregular spaces lined by epithelium. Another form is found in the neonate and involves large portions or all of a lung. This is associated with a developmental aberration and is best described as a "congenital adenomatoid malformation". An apparently unique noninvasive tumor mass was resected from the lung of a middle aged man where it was associated with anomalous lung segmentation and the bronchial and blood supply to the lung. The tumor appeared to be undifferentiated by light microscopic criteria. Neurilemoma, leiomyoma, chordoid tumor, mixed tumor, and neurogenic sarcoma all entered the differential diagnosis. Ultrastructural examination demonstrated a highly complex and unique organization. Such a lesion, apparently developmental in this case, should be recognized and carefully distinguished from the malignant mesenchymal, neurogenic, and teratomatous lesions with which it may be confused. Electron microscopy may be helpful in this regard.

Acute myelofibrosis in progressive systemic sclerosis. Report of a case developing during long term chlorambucil therapy and discussion of pathogenetic factors.

A case of acute myelofibrosis occurring in the course of long-term chlorambucil therapy for cardiac manifestations of progressive systemic sclerosis (PSS) is reported. Although hematologic malignancies have been known to develop consequent to long-term use of alkylating agents, and bone marrow suppression is well known, acute myelofibrosis has not, to our knowledge, been reported as a complication of this therapy, nor has any specific myeloproliferative syndrome been described consequent to such therapy in a patient with PSS. As abnormal fibroblastic proliferation is central to both PSS and myelofibrosis, it may be speculated that the patient with scleroderma may be predisposed to this particular expression of hematologic dyscrasia. The roles of fibroblastic proliferation, and immunologic and vascular factors in these two illnesses are currently poorly understood.

Unusual variant of lipid-rich mammary carcinoma.

A variant of lipid-secreting mammary carcinoma shared many of the features of previously reported lipid-rich breast tumors, ultrastructurally as well as clinically and by light microscopy. In addition, electron microscopy confirmed the presence of a dual-cell population where the more aggressive cell, which resembled an altered myoepithelial cell, was responsible for the lipid production. The tumor also demonstrated some similarity to medullary carcinoma and may represent a variant of this type of mammary tumor.

Mycosis fungoides-like cells. Their presence in a case of pityriasic dermatitis with a comment on their significance as an indicator of primary T-cell dyscrasia.

A case of pityriasic dermatitis in which the histologic findings mimicked mycosis fungoides was examined ultrastructurally. Large numbers of mycosis-like cells were found in the dermis and within epidermal spongiotic vesicles. Such cells occur in the epidermal and dermal infiltrates of primary T-lymphocyte disorders, notably in mycosis fungoides, the Sézary syndrome, and parapsoriasis en plague. However, they have also been found in the dermal infiltrates of benign dermatoses, in some skin tumors, and occasionally in normal controls. They share ultrastructural features with transformed T lymphocytes. It is emphasized that cells with this morphology may be found in the skin in any condition involving T-cell transformation or dysplasia. The mere presence of cells with this morphology within skin infiltrates is insufficient evidence for the diagnosis of primary T-cell dyscrasia.

Squamous cell carcinoma of the tongue in a nine year renal transplant survivor: a case report with a discussion of the risk of development of epithelial carcinomas in renal transplant survivors.

A case of squamous cell carcinoma of the tongue in a 26-year-old man developing nine years after renal transplantation is presented. This is the first such case to be reported. Within seven months, widespread tumor metastases resulted in death. Pathologic examination of the transplanted kidney demonstrated neither re-establishment of glomerulonephritis nor evidence of rejection, and lack of significant renal disease was confirmed by electron microscopy. This long term survival without development of renal pathology is also of great interest.

Neurilemoma-like uterine myomas: an ultrastructural reaffirmation of their non-Schwannian nature.

Some uterine leiomyomas demonstrate a curious rhythmic pattern of cellular arrangement, suggesting the appearance of nerve sheath tumors. Leiomyomas of similar appearance are more frequently found in the gastrointestinal tract. Ultrastructural examination of two such tumors from the uterus, one simulating the Antoni type A pattern and the other predominantly of the Antoni type B pattern, showed features of smooth muscle cells to be present in both. There also was a close fine structural resemblance to light microscopically similar tumors of the gastrointestinal tract. This study should dispel the implications that these tumors are of Schwannian origin.

Adenocarcinoma of the rete testis: a review of the literature and presentation of a case with associated asbestosis.

Although scattered reports of adenocarcinoma of the rete testis have previously appeared and criteria for the diagnosis of this tumor have been defined, previous cases have not been described in sufficient detail to provide an organized account of the nature and behavior of this neoplasm. The present report describes a case followed to autopsy and provides conclusive evidence that it is of rete testis origin. Previously reported cases of rete testis carcinoma are reviewed and the salient features summarized, showing that the tumor has a definite clinical and histologic pattern and that the prognosis appears poor. The present case occurred in a patient with severe pulmonary asbestosis. This association is unique, and its significance remains speculative.