Recurrent angiomatoid fibrous histiocytoma: a case report and review of the literature.

BACKGROUND: Angiomatoid fibrous histiocytoma (AFH) is a rare soft-tissue tumor with intermediate malignant potential. It most commonly occurs in children and young adults. CASE REPORT: We report a case of a recurrent AFH of the back, locally recurring within 14 months of the original operation. We also review the literature for this uncommon entity. The patient underwent a wide resection of the recurrent AFH, obtaining negative surgical margins. Postoperatively, he has done well. CONCLUSION: Twelve months since his re-excision, the patient shows no evidence of recurrence to date. He is seen every 6 months for continued clinical examinations.

Primary amyloidosis of the breast presenting as a solitary nodule: case report and review of the literature.

BACKGROUND: Amyloidosis of the breast is an unusual diagnosis. Overall, only 2 major case series have been published in the literature since the time amyloidosis was first reported in 1973, highlighting the rare nature of this disease. Clinically, the patient may present with a breast mass or simply increased breast density and skin thickening. As such, amyloidosis may mimic the appearance of a number of pathologies, both benign and malignant. CASE REPORT: We report a single case of a patient who presented with a breast mass and was ultimately diagnosed with primary amyloidosis of the breast. We also present a review of the literature to date. CONCLUSION: The main treatment for primary amyloidosis of the breast is surgical removal, most often accomplished with breast conservation via lumpectomy. In performing the lumpectomy, the goal should be to obtain negative surgical margins; however, no strong data exist to suggest that such lesions would recur with a microscopically positive margin.

Lacrimal sac melanoma.

A 48-year-old woman presented with a 5-month history of unilateral epiphora and progressive growth of a left-sided medial canthus mass. CT revealed a nonenhancing soft tissue mass of the left lacrimal sac with widening and erosion of the nasolacrimal canal. The tumor was resected and pathologically confirmed to be malignant melanoma. Despite wide resection and adjuvant radiotherapy, the patient developed metastases to the chest wall. The patient currently is receiving combined immunotherapy and chemotherapy. The clinical course and treatment strategies are reviewed for this highly aggressive and rare neoplasm.

Immunostaining for SYT protein discriminates synovial sarcoma from other soft tissue tumors: analysis of 146 cases.

Synovial sarcoma in its classic biphasic form can be distinguished readily from other soft tissue lesions; however, monophasic and poorly differentiated forms are diagnostically more problematic. For this reason, we assessed the efficacy of immunostaining for SYT and SSX1 proteins, the gene products resulting from unique synovial sarcoma translocation, to distinguish synovial sarcoma from other soft tissue lesions. A total number of 146 cases were analyzed, including 47 synovial sarcoma cases (all of which were verified by FISH to have t(X; 18) translocation and SYT-SSX fusion gene) and 99 soft tissue tumors of various types. A polyclonal IgG antibody against SYT was used to stain formalin-fixed paraffin embedded tissues. Forty-one out of 47 (87%) synovial sarcoma displayed strong positive nuclear staining (ranging from 80 to 90% of the tumor cells) for SYT antibody. Nineteen of 99 (19%) non-synovial sarcoma cases showed variable nuclear and cytoplasmic staining with SYT, which ranged from 20 to 60% of tumor nuclei, and included malignant peripheral nerve sheath tumor (5/25), solitary fibrous tumor (2/14), Ewing sarcoma (2/6), low grade fibromyxoid tumor (2/4), extraskeletal mesenchymal chondrosarcoma (2/6), gastrointestinal tumor (4/17), epithelioid sarcoma (2/2). The remaining non-synovial sarcomas were negative. This is the first study demonstrating SYT protein expression in tissue sections of synovial sarcoma. This method could provide an easy, rapid and widely applicable means of assisting in the diagnosis of synovial sarcoma, particularly when material and/or resources are unavailable for PCR or FISH-based testing. However, as variable weak staining for SYT may be encountered in a small percentage of non-synovial sarcoma sarcomas, a positive interpretation should be made only when the staining is strong, nuclear and present in the majority of cells.

Monostotic fibrous dysplasia.

Fibrous dysplasia is most often monostotic and an incidental finding in an asymptomatic patient. Familiarity with the disease can promote early diagnosis and avoid lengthy evaluation. The polyostotic form of the disease and its variants, however, can have significant impact on the patient and is associated with several well-defined syndromes. Imaging is central for diagnosis and treatment planning. A wide spectrum of imaging findings includes the characteristic "ground-glass" pattern of mineralization and medullary expansion. Surgical intervention may be necessary to manage secondary complications and deformities.

Report of an angiosarcoma mimic: cutaneous aneurysmal fibrous histiocytoma.

Aneurysmal fibrous histiocytoma is an unusual variant of the spectrum of fibrous histiocytomas with the peculiar morphologic appearance of a benign aneurysmal vasoformative process that ultimately culminates in multiple microhemorrhages within the tumor. It looks strikingly different from the usual cutaneous lesions encountered in clinical dermatology practice. A single report of a cutaneous aneurysmal fibrous histiocytoma in the skin of the back of a 60-year-old male is described with emphasis on the immunostaining pattern and review of the literature. There is a significant potential for confusion of this lesion with other cutaneous lesions, clinically as well as pathologically. In our case, the patient presented with a lesion that clinically resembled a hemangioma, was pathologically interpreted initially to be an angiosarcoma, and finally, the revised pathology was interpreted as an aneurysmal variant of a fibrous histiocytoma. Caution is warranted to avoid misinterpretation of cutaneous fibrohistiocytic tumors.

Clear cell sarcoma of the small bowel: a potential pitfall. Case report.

Clear cell sarcoma (soft-part melanoma) is a very rare entity with a distinctive histopathologic and molecular profile. Herein, we present the sixth reported case of a primary gastrointestinal clear cell sarcoma discovered in a 21-year-old woman. The patient underwent numerous tests prior to the diagnosis of her small bowel pathology, including the use of capsule endoscopy, which allowed for visualization and final localization of the tumour. Additionally, we discuss this rare type of sarcoma that affects young adults and has a poor prognosis characterized by the balanced chromosomal translocation t(12;22)(q13;q12) with special emphasis on the necessity for pathologists to be able to distinguish it from melanoma -- potentially a major pitfall in diagnosis.

Mesonephric adenocarcinoma of the vagina with a 3-year follow-up.

BACKGROUND: Mesonephric adenocarcinoma of the vagina is exceedingly rare, with only one well-documented case in the literature. Little is known regarding clinical presentation, pathological characteristics, therapy, or prognosis of the vaginal mesonephric adenocarcinoma. CASE: A 55-year-old woman presented with a polypoid mass at the right vaginal apex, extending to the right paravaginal tissue. The tumor was an adenocarcinoma with ductal and tubular pattern arising in a background of mesonephric remnants. Tumor cells showed immunoreactivity for pankeratin, epithelial membrane antigen (EMA), and calretinin. The right fallopian tube and one paravaginal lymph node were positive for metastases. The patient is disease-free 3 years after surgery, radiation therapy, and chemotherapy. CONCLUSION: We report the second case of mesonephric adenocarcinoma of the vagina with metastasis to the right fallopian tube and to one paravaginal lymph node.

Controlled myocardial infarction induced by intracoronary injection of n-butyl cyanoacrylatein dogs: a feasibility study.

Concentrated ethanol has been used to induce controlled myocardial infarct in patients with hypertrophic obstructive cardiomyopathy. We report the acute and early follow-up results of an alternative agent, n-butyl cyanoacrylate (n-BCA) glue, in a dog model. In 11 mongrel dogs, we injected n-BCA into different branches of the left anterior descending artery. Biplane left ventriculogram and coronary angiogram were performed before and after injection. In the surviving animals, we performed programmed stimulation (PS) to test for inducible ventricular tachycardia (VT) 48 days later. Following euthanasia, the removed hearts were studied with computer tomography (CT) and gross and histologic examination. Three dogs were lost before injection. Four dogs died within 2 hr to 4 days, and four animals survived 48 days. Accidental embolization of n-BCA into nontarget vessels was documented in four subjects. In the n-BCA-injected animals, homogeneous circumscribed scar was demonstrated by CT and histology. The glue was confined strictly to the tributary of the injected vessel, infiltrating arterioles of 14 mum. There was intense granulomatous reaction (GR) in the vessel wall and in the surrounding myocardium. Remote areas were unaffected. Monomorphic VT was not inducible with PS. We report a feasibility study of n-BCA injection to selected coronary arteries of dogs to cause controlled myocardial infarction. We demonstrated that the glue does not escape from the target artery through capillaries or small collateral vessels and thus produces a sharply demarcated and homogeneous scar, which is confined strictly to the supply zone of the injected vessel. Improvement of the delivery system is necessary to eliminate inadvertent embolization. Long-term follow-up is needed to study the GR induced by n-BCA.

Metastatic juxtaglomerular cell tumor in a 52-year-old man.

Juxtaglomerular cell tumor is a rare renal neoplasm arising from the juxtaglomerular apparatus. Approximately 70 cases have been reported in the English literature since it was first described by Robertson et al in 1967. This tumor has been considered benign and resection has so far been curative. In this paper, we report the first metastatic juxtaglomerular cell tumor. The 15-cm tumor occurred in the right kidney of a 46-year-old man. It invaded the renal vein, and was treated by radical nephrectomy in 1995. The diagnosis at that time was renal cell carcinoma. The patient was well for 6 years and then developed bilateral lung masses, which were resected. Microscopically, the tumors from the kidney and the lungs were similar, consisting of solid sheets of uniformly round-to-polygonal cells intermixed with abundant delicate vasculature. Both renal and pulmonary tumors were positive for vimentin, renin, and only focally to CD34. Electron microscopic studies performed on the paraffin-embedded renal tumor and formalin-fixed lung tumor revealed the typical rhomboid crystals of proto-renin. In consideration of the characteristic morphologic features, immunohistochemistry, and the presence of rhomboid crystals of proto-renin, the diagnosis was modified to malignant juxtaglomerular cell tumor.