Sinonasal undifferentiated carcinoma: a 10-year experience.

PURPOSE: Sinonasal undifferentiated carcinoma (SNUC) is a rare and aggressive malignancy of the paranasal sinuses and nasal cavity. Of the few reported series, most indicate a dismal prognosis. In this report, the clinical presentation, histopathologic criteria used for diagnosis, mode of treatment, and outcome are evaluated in seven patients with SNUC. MATERIALS AND METHODS: Seven patients with SNUC treated at the University of Cincinnati between 1983 and 1993 were analyzed retrospectively. RESULTS: Most of the patients presented with extensive local disease, and two patients also had cervical metastases. All except one were treated using a multimodality approach. Four of the seven patients died of disease (DOD), with a mean survival of only 11.5 months following treatment. Inability to eradicate local disease was responsible for treatment failure in all cases. Three patients have achieved short-term control of disease following combined therapy, but one is at high risk for recurrence. CONCLUSION: SNUC was associated with an overall poor prognosis in our series despite aggressive treatment. Control of local disease was the central therapeutic consideration. Intensive multimodality therapy is recommended for all patients with SNUC.

Simple cyst of the testis.

We report 5 cases of simple cyst of the testis, the largest series to date, and review the literature. Included are 2 autopsy cases, a case of a multilocular simple cyst and 1 case treated nonoperatively. Based on our experience, simple cyst of the testis should be suspected particularly in an enlarged but otherwise palpably normal testis. High resolution real-time scrotal sonography revealing an anechoic mass in the testis with well defined walls, and enhanced posterior through transmission and edge shadowing is diagnostic for a simple cyst. An asymptomatic simple cyst of the testis may be treated with observation alone. The symptomatic simple cyst should be treated ideally with local parenchyma-sparing excision.

Benign Mucor colonization (fungus ball) associated with chronic sinusitis.

Mucormycosis of the nasal cavity and paranasal sinuses is an aggressive, life-threatening condition characterized by fulminant destruction of soft tissues and bone, vascular invasion, and thrombosis. Patients who have this infection generally are immunosuppressed or diabetic. The most effective treatment is immediate surgical debridement of all involved tissue, along with systemic antifungal agents. We identified four patients with chronic sinusitis in whom Mucor organisms were histologically confirmed within the sinus contents but not in the sinus mucosa. All four were successfully treated with conservative debridement. Our dilemma was determining appropriate treatment for this apparently less aggressive condition. We conclude that conservative debridement is the treatment of choice in the absence of tissue invasion, and that systemic antifungal therapy is probably unnecessary.

Malignant lymphoma presenting as a renal mass: four cases.

Primary lymphoma of the kidney is extremely rare; most lymphomatous renal masses represent extension from adjacent sites of disease or involvement by generalized disease (4,9,12). Three men and one woman, 45 to 71 years of age, presented with solitary renal masses clinically thought to be renal cell carcinoma. Each experienced abdominal pain, one with hematuria and one with "B" symptoms. Physical examination revealed no peripheral lymphadenopathy or hepatosplenomegaly. Lactic dehydrogenase (LDH) was elevated in three cases, and blood urea nitrogen (BUN) and creatinine were slightly increased in two. Two cases were diagnosed correctly from needle biopsy, with ultrastructural confirmation in one case and marker studies, DNA flow cytometry, and cytogenetics in the other. Because of a presumptive diagnosis of renal cell carcinoma, two patients underwent nephrectomy. Three cases were large-cell lymphoma, and one, small noncleaved cell lymphoma.

Upper aerodigestive tract metastases in disseminated malignant melanoma.

Cutaneous malignant melanoma sometimes metastasizes to the upper respiratory and digestive tracts. It may cause significant local symptoms such as airway obstruction and dysphagia, and, in some cases, may represent the initial manifestation of disseminated disease. Of the 8,823 patients with cutaneous malignant melanoma seen at The University of Texas-M.D. Anderson Hospital and Tumor Institute at Houston between 1944 and 1983, metastases to this region developed in 54 patients. The most common sites involved were the tonsil, tongue, nasopharynx, larynx, and lip. Five of ten cases in which an autopsy was performed were noted to have previously undiagnosed metastatic mucosal lesions. We conclude that metastases to the upper aerodigestive tract in patients with cutaneous malignant melanoma is a distinct possibility. Melanoma patients who manifest symptoms localized to this region should be carefully examined to exclude the possibility of metastatic tumor, since alternative treatment may be required. Local endoscopic treatment may be necessary to relieve airway or digestive tract obstruction.

Osseous choristoma of the tongue.

I have reported three cases of asymptomatic osseous choristoma of the tongue and reviewed the 38 previously reported cases, 80% of which occurred in women and in patients less than 40 years old. Most osseous choristomas are located in the posterior third of the tongue and primarily in the vicinity of the foramen cecum and/or circumvallate papillae. Derivation from a remnant of the cartilaginous matrix of the second and third branchial arches is the favored hypothesis of origin. Simple excision is adequate. None has recurred.

Metastatic carcinoma of breast in the hypopharynx.

We have described a case of multiple metastases in the hypopharynx found at autopsy in a 43-year-old woman with carcinoma of the breast. No symptoms related to the hypopharyngeal lesions were recorded. A metastasis, although rare, should be included in the differential diagnosis of a hypopharyngeal lesion since it may clinically mimic a benign neoplasm or primary carcinoma.

Ectopic salivary gland tissue in submucosa of rectum.

A case of ectopic salivary gland tissue in the submucosa of the rectum adjacent to a hyperplastic polyp is described. The two previously reported cases of ectopic salivary gland tissue in the rectum were different in that ectopic gastric mucosa was also present. The 16 cases of ectopic tissue in the rectum noted in the world medical literature are reviewed.

Adenocarcinoma of prostate in a twenty-seven-year-old man.

A case of adenocarcinoma of the prostate in a twenty-seven-year-old man is reported. He responded initially to cobalt radiation to the prostate, but metastases developed subequently and he died thriteen months after the histologic diagnosis was made. Three of the 4 previously documented cases in men below age thirty who also presented with symptoms of obstruction of the lower urinary tract or bone pain died four, five, and twelve months later. The other patient was asymptomatic and alive thirty-one months after detection on a routine physical examination. Although based on a small number of cases, men below age thirty with prostatic adenocarcinoma have a poor prognosis, which appears to be related to the presence of symptoms at the time of diagnosis.

Plexiform neurofibroma of major salivary glands in children.

Two cases of plexiform neurofibroma of major salivary glands in children are presented. The sites were the sublingual glands in one and the parotid and submaxillary glands in the other. The four previously reported cases in children, all involving the parotid, are briefly reviewed. A sarcomatous component was present in one case. Although plexiform neurofibroma of the major salivary glands is unusual, the possibility should be considered in those patients with von Recklinghausen's disease who develop a major salivary gland tumor.

Leiomyosarcoma of the anterior maxillary alveolar ridge.

A case of leiomyosarcoma of the anterior maxillary alveolar ridge, including ultrastructural examination findings, in a 39-year-old woman is reported. The patient was clinically free of tumor 34 months later. The twenty-three previously reported cases of oral leiomyosarcoma are reviewed briefly.

Postpartum dissecting aneurysm of coronary arteries in a patient with sarcoidosis.

We have described a case of sudden death in a 27-year-old woman, 16 days postpartum, resulting from a dissecting aneurysm of the right and left coronary arteries. The patient also had multiorgan sarcoidosis. Postpartum coronary artery dissecting aneurysm appears to be caused by weakening of the media, resulting from the loosening of the ground substance during pregnancy and the hemodynamic changes occurring during and after delivery.

Retroperitoneal lipoma in children.

A case of retroperitoneal lipoma in a 23-mo old girl is reported together with a brief review of the 11 previously documented cases. It has been encountered through age 8 yr and twice as frequently in girls. The majority had an enlarged abdomen of long duration. The others were asymptomatic and found incidentally or at the time of examination for abdominal pain. Most lipomata were large. The possibility of their being congenital cannot be excluded. None have recurred.

Adult rhabdomyoma of soft palate.

A case of adult rhabdomyoma of the soft palate in a 60-year-old man studied by methods including electron microscopy, is reported. The patient was free of recurrence 2 years later. The eleven previously documented cases of adult rhabdomyoma involving the oral cavity are reviewed. The concept that rhabdomyoma is a neoplasm rather than a hamartoma is favored. Adequate local excision appears to be curative. The differences between the cardiac and extracardiac forms of rhabdomyoma as well as between adult rhabdomyoma and especially granular-cell myoblastoma and fetal rhabdomyoma are presented.