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A 70-year-old man had an obstruction of the aortic bifurcation by a primary aortic sarcoma. The tumor was not fibrosarcomatous, as are the majority of primary aortic tumors. To our knowledge, this is the third occurrence of this type of primary aortic malignant neoplasm to be reported in the literature. A possible endotheliomatous origin is suggested.
Of 148 patients treated for abdominal aortic aneurysms (AAA), 33 (22%) also had cancer. According to the classification of Szilagyi, there were 13 patients in group I, 19 in group II, and 1 in group IV. In group I, the mean interval between the cancer and AAA operations was 7 years (range 1-14 years). Aneurysmectomy was performed in 9 patients, wrapping in 2, and no operation in 2. In group II, a two-stage operation was performed in 8 patients, a single-stage operation in 4, only surgery for cancer in 4, and no operation in 3. Of 4 patients undergoing single-stage operations, 3 had colorectal cancer, and there were no postoperative complications such as graft infection or anastomotic breakdown. In group I, 6 of 13 patients died, but there were no cancer deaths. In group II, 9 of 19 patients died, 6 from progressive cancer. The group IV patient also died of cancer. These results suggest that if a patient can tolerate surgery for both diseases, a single-stage operation is preferable.
A case of angiomyofibroblastoma-like tumor (lipomatous variant) that arose in the subcutis of the inguinal region of a 45-year-old man is presented. The maximum dimension of the tumor measured 39 mm and it was well circumscribed. Histopathological examination revealed a close admixture of plump spindle or stellate cells and mature adipocytes. These were arranged haphazardly or formed interconnecting thin cellular trabeculae on the myxoid or edematous background. The tumor was highly vascularized and contained a moderate amount of collagen fibers. Immunohistochemically, the tumor cells were positive for vimentin, CD34 and the estrogen receptor, but negative for alpha-smooth muscle actin and desmin. The differential diagnosis between the lipomatous variant of angiomyofibroblastoma and cellular angiofibroma or spindle cell lipoma is discussed, and the pathogenetic hypotheses about the occurrence of mature adipocytes within benign fibroblastic or myofibroblastic neoplasms are mentioned briefly. It is important for surgical pathologists to be acquainted with the occasional presence of adipocytes in these neoplasms to avoid misinterpretation.
CT of the body has had major impact in the diagnosis of trauma, infection, and neoplasms in children. Properly used, it is a safe, accurate, and cost-effective imaging procedure which can replace many previously required conventional imaging studies.
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BACKGROUND: Granulocytic sarcoma (GS) is the rare extramedullary manifestation of acute myeloid leukemia that may precede or be concurrent with leukemic infiltration of bone marrow or herald blastic transformation of a chronic myeloproliferative disorder. It has been found in most body sites and shows no age or sex predilection, necessitating its inclusion in the differential diagnosis of undifferentiated neoplasms. CASE: A 36-year-old female presented with a three-year history of abdominal pain, jaundice and fluctuating abdominal girth. Cytology of the ascitic fluid revealed myeloid cells of eosinophilic lineage at all stages of differentiation, with many undifferentiated cells. Immunohistochemical studies on a cell block confirmed the diagnosis of granulocytic sarcoma, which excluded the differential diagnoses of Hodgkin's disease, non-Hodgkin's lymphoma and Langerhans histiocytosis. CONCLUSION: Granulocytic sarcoma may present as a serous effusion and can be diagnosed on a cytologic specimen.
A straightforward method of tissue analysis from dual-energy computed tomography (CT) is presented which does not rely on previous inaccurate or incorrect formulations of the X-ray attenuation coefficient. The attenuation coefficients of tissue and bone were represented by a mixture of two reference materials. For convenience, water and calcium chloride were chosen. After careful calibration of the CT scanner, a dual-energy CT scan yields the water and calcium chloride in units of kg/kg multiplied by the specific gravity of the tissue. For an error of +/- 2 HU on dual-energy scans at 140 kVp and 87 kVp on an EMI CT5005 general-purpose scanner, the error on the calcium chloride coefficients is +/- 0.004 kg/kg. Fat concentrations greater than 25% by weight may be detected when the coefficients are averaged over at least 200 pixels. Bone mineral content could be measured with a precision of 0.01 (in units of kg/kg multiplied by specific gravity) for averaging regions of 24 pixels. Iodine concentrations in tissue can be deduced if water and iodine are chosen as the reference materials. Clinical examples are presented to illustrate the technique for scans of both the head and the abdomen.
OBJECTIVE: The radiologist and oncologist are often confident that biopsy will confirm their suspicion of recurrent disease, but a biopsy is performed to confirm the histologic diagnosis before beginning or altering therapy. We have examined data to determine how often the biopsied lesion represents recurrent disease from the primary tumor or is an instance of new cancer, and whether recurrent disease can be predicted. MATERIALS AND METHODS: We reviewed the medical and imaging records of 253 patients who underwent CT-guided biopsy of an abdominal or pelvic lesion between 1993 and 1996. Sixty-nine of the 253 patients had a previously diagnosed primary tumor and were being examined for possible tumor recurrence or metastasis. The images of these 69 patients were analyzed to determine if the pattern of disease was typical of recurrence or metastasis. RESULTS: In 55 of the 69 patients, the pattern was judged to be typical of metastatic or recurrent disease. Biopsy confirmed this suspicion in all 55 patients. In 14 of the 69 patients, the pattern of spread was judged not to be typical of recurrence or metastasis. These 14 patients were found to have a new primary tumor (n = 4), benign processes (n = 2), and recurrences (n = 8). CONCLUSION: Of the patients for whom radiographic findings suggested recurrence, we found no patients in whom a new primary tumor would have been missed if biopsy had been avoided. Data should now be acquired prospectively to determine whether it may be prudent to make treatment decisions on the basis of imaging findings alone, without histologic confirmation.
Desmoplastic small round cell tumour (DSRCT) is a unique, highly aggressive neoplasm that chiefly affects adolescent male and young adults and most frequently presents as a large abdominal mass with widespread peritoneal involvement at the time of diagnosis. Histologically, it is composed of nests of small, undifferentiated round or oval hyperchromatic cells embedded in abundant desmoplastic stroma. Co-expression of epithelial, mesenchymal, and neural antigens in the same cell provides evidence of origin from a primitive pluripotent stem cell with multiphenotypic differentiation. A multidisciplinary treatment including high-dose chemotherapy, aggressive debulking surgery, radiation and myeloablative chemotherapy with stem cell rescue might be the proper approach to treat this rare malignancy and may improve progression-free survival.
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The clinical, laboratory, radiographic and pathological findings of two dogs with similar neoplasms are described. Both dogs were mature males of a large breed and had pleural and abdominal effusions containing neoplastic cells. Diffuse scirrhous thickening of the pleura and serosal surfaces was a prominent feature. A fibrous layer containing nests of neoplastic, epithelial-like cells was present on serosal surfaces. The morphological appearance of the cells was suggestive of either an atypical sclerosing mesothelioma or a scirrhous carcinoma. On the basis of ultrastructural and differential staining characteristics, a diagnosis of a scirrhous carcinoma of undetermined primary origin was made in one case and of prostatic urethral origin in the other case.
Giant cell angiofibroma is a rare mesenchymal neoplasm most commonly arising in the soft tissues near the orbit. Recently, several cases of extraorbital giant cell angiofibroma have been reported. We report the light microscopic and immunohistochemical features of an additional case of extraorbital giant cell angiofibroma arising in the inguinal region that was clinically mistaken for an inguinal hernia. The patient was a 50-year-old woman who presented with a mobile, nonreducible, left inguinal mass. The tumor was 10.8 cm in greatest diameter, was well circumscribed, and appeared to be encapsulated. Histologically, the tumor was composed of a mixture of cytologically bland spindle-shaped cells and ovoid cells of varying cellularity with deposition in a variably collagenous and myxoid stroma. The tumor had prominent, various-sized blood vessels, often with perivascular hyalinization. In addition, scattered pseudovascular spaces filled with an amorphous eosinophilic material were present and lined by spindle-shaped and ovoid cells similar to those found throughout the neoplasm. Rare multinucleated floret-like giant cells were seen. Immunohistochemically, the tumor cells stained strongly and diffusely for both CD34 and bcl-2 while immunostains for S-100 protein, desmin, smooth muscle actin, and muscle-specific actin were negative. There is no evidence of local recurrence or metastasis 3 months following excision of the mass. This report emphasizes the recognition of this unusual tumor in extraorbital sites. We discuss the overlapping histologic and immunophenotypic features with giant cell fibroblastoma and solitary fibrous tumor and raise the possibility that these tumors could represent a histologic spectrum of CD34-positive dendritic interstitial cell neoplasms.
The authors study 39 cases of late genitourinary sequelae following abdominopelvic radiotherapy. Several points appear to be of interest: - the mean latent period was 3.5 years for bladder sequelae and 4 years for ureteric complications. Fistulae could develop on average 14 years after radiotherapy; - the possibility of ureteric complications in the form of stenosis after radiotherapy, exclusive of associated surgery; - all lesions were purely due to radiation, excluding recurrences of neoplasm; - bladder complications had striking symptoms with dysuria and haematuria. Ureteric stenosis was sometimes found by chance, being latent; - associated digestive tract radiation lesions were seen in 1 case in 4; - the frequent need for surgery, since this was required in 30 patients out of 39. The operations performed were often extensive; - the grave nature of surgical operations including a digestive phase in these patients who had received large doses of radiation. Peritonitis due to the breakdown of sutures was a notable source of mortality; - one of the great difficulties in investigating such patients is to be able to confirm the purely radiation aetiology of these complications, bearing in mind that amongst urological complications following the treatment of pelvic carcinomas, the majority are related not only to radiation but also to recurrence of neoplasm.
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