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Juvenile elastoma: a forme fruste of the Buschke-Ollendorff syndrome?

We describe an Asian child with juvenile elastoma who presented to us at 9 years of age with flesh-coloured nodules on his thigh. Histologically, H&E sections were normal, but elastin stains showed collections of increased and thickened elastic fibres consistent with juvenile elastoma. Radiological examination did not demonstrate osteopoikilosis. However, we postulate that he may represent a forme fruste of the Buschke-Ollendorff syndrome or early presentation prior to the subsequent development of osteopoikilosis.

Child↗

Malignant cartilaginous tumors of the mediastinum: clinicopathological study of six cases presenting as extraskeletal soft tissue masses.

Cartilage and bone-forming tumors of the mediastinum are extremely rare neoplasms with very few cases having been reported in the literature. We studied six cases of primary malignant cartilaginous tumors presenting as extraskeletal soft tissue masses in the posterior mediastinum. The patients were five women and one man aged 11 to 63 years (median, 31 years). Histologically, the lesions showed a spectrum of features that ranged from mesenchymal chondrosarcoma, to extraskeletal myxoid chondrosarcoma, to moderately well to poorly differentiated chondrosarcoma. In all cases, the lesions presented as well-circumscribed tumor masses centered in the soft tissues in the posterior mediastinum without radiographic evidence of origin from bone. Because of their relatively small size, good circumscription, focal areas of calcification, and posterior mediastinal location, the preoperative clinical diagnoses included benign neurogenic tumor and neuroblastoma. All of the lesions were treated by complete surgical excision, followed in two cases by postoperative radiation therapy. Clinical follow-up was available in five cases: two patients with mesenchymal chondrosarcoma presented with local recurrence after 3 and 7 years, one developed metastases to the sacrum 8 years after initial diagnosis and died, and one was alive and well without evidence of disease after 6 years. The patient with myxoid chondrosarcoma of the posterior mediastinum developed bilateral pulmonary metastases 10 months after surgery and has been lost to follow-up since. Our findings reinforce previous observations on the occurrence of extraskeletal cartilaginous tumors in the mediastinum and indicate that these tumors can show a propensity for local aggressive behavior with high recurrence rate and a definite potential for distant metastases. Such tumors should be considered in the differential diagnosis of malignant neoplasms presenting as a soft tissue mass in the posterior mediastinum.

Adolescent↗

Heterotransplantation of cultured human cancer cells and human cancer tissues into nude mice.

Twenty-seven out of 31 cultured human cancer cell lines transplanted subcutaneously into nude mice produced solid tumors. Direct transplantation of surgical materials proved less successful. In 35 attempts, only 1 of 6 gastric cancers, 2 of 3 liposarcomas, and 1 of 3 osteosarcomas were accepted. No positive tumor formation was noted after the inoculation of 3 nasopharyngeal carcinomas or 1 breast cancer. None of lymphomatous neoplasms and leukemias produced any tumor in 12 and 3 attempts, respectively. The histological and cytological characteristics of the tumors developed were studied with light and electron microscopes, in relation to the features in vitro and those of the parent tumors. Histological similarity of the tumors that developed in nude mice to their parent tumors were noteworthy. Serial transplantation was performed successfully in 9 cell lines and 2 tissues. Preservation of tissue construction ability and other differentiation abilities of in vitro cultured human cancer cells were ascertained in varying degrees.

Adenocarcinoma↗

Feasibility of autonomic nerve-preserving surgery for advanced rectal cancer based on analysis of micrometastases.

BACKGROUND: Autonomic nerve preservation has been advocated as a means of preserving urinary and sexual function after surgery for rectal cancer, but may compromise tumour clearance. The aim of this study was to determine the incidence of micrometastasis in the connective tissues surrounding the pelvic plexus. METHODS: The study included 20 consecutive patients who underwent rectal surgery with bilateral lymph node dissection for advanced cancer. A total of 78 connective tissues medial and lateral to the pelvic plexus and 387 lymph nodes were sampled during surgery. All connective tissue samples and 260 lymph nodes were examined for micrometastases by reverse transcriptase-polymerase chain reaction (RT-PCR) after operation. All patients were followed prospectively for a median of 36.0 months. RESULTS: Of 245 histologically negative lymph nodes, 38 (15.5 per cent) were shown by RT-PCR to harbour micrometastases. However, micrometastases to tissues surrounding the pelvic plexus were detected in only two (3 per cent) of 78 tissues, that is in two of 20 patients. Clinical follow-up showed that the two patients had a poor prognosis owing to distant metastases. CONCLUSION: Autonomic nerve-preserving surgery may be feasible for advanced rectal cancer, but study of more patients positive for micrometastases is required.

Adult↗

Immunohistochemical studies in the differential diagnosis of small round cell soft tissue tumors.

In this review the authors focus on the differential diagnosis of small round cell tumors of soft tissue origin, giving emphasis on their specific immunohistochemical profiles. The sources of diagnostic errors and misinterpretations of the results of immunohistochemical staining are discussed in detail. It is thorough knowledge of specific characteristics of each tumor and appropriate interpretation of different staining techniques that will lead to precise classification of any given tumor, thus allowing accurate determination of prognosis and implementation of the optimal treatment.

Biomarkers, Tumor↗

Chondromyxoid fibroma of the nasal bone with extension into the frontal and ethmoidal sinuses: report of one case and a review of the literature.

Chondromyxoid fibroma is a rare benign tumor that usually occurs in the long bones. A 50-year-old patient presented with chondromyxoid fibroma of the nasal bone with extension into the frontal and ethmoidal sinuses. This is the fourth case reported to date in the literature. The clinical manifestations of the tumor were very limited, and the appearance at rhinoscopy was misleading. Radiologic imaging showed a soft tissue lesion invading the adjacent bony structures and the dura mater. Surgery was performed by a combined team of otorhinolaryngologists and neurosurgeons, and total excision of the tumor was achieved. The histologic diagnosis of this tumor is difficult because of its similarities to chondrosarcoma. [Editorial comment: The authors concisely review management of this rare tumor, emphasizing that complete surgical excision, rather than curettage, is required for long term control.]

Ethmoid Sinus↗

Ectomesenchymal chondromyxoid tumor of the anterior tongue. Nineteen cases of a new clinicopathologic entity.

We present 19 cases of a previously undescribed myxoid tumor of the anterior tongue. These lesions occurred in nine women and 10 men aged 9 to 78 years (median, 32 years). Most tumors were seen as slow growing, painless nodules in the anterior dorsal tongue. The duration of growth ranged from a few months to 10 years. All tumors were treated by surgical excision, and two recurred. Microscopically, they exhibited a lobular proliferation of ovoid and fusiform cells, which often had multilobated nuclei and occasional foci of atypia, in a chondromyxoid background. Some tumors entrapped muscle or nerve fibers and had a tendency for blunt infiltration of adjacent tissue. The cells were diffusely and intensely immunoreactive for glial fibrillary acidic protein (GFAP) and cytokeratin but were decorated less frequently with antibodies for smooth muscle actin and S-100 protein. Reactivity for epithelial membrane antigen and desmin was not found. We believe these tumors fail to meet established clinicopathologic criteria for any existing myxoid neoplasms of the tongue, including nerve sheath myxoma, myoepithelioma, benign mixed tumor, ossifying fibromyxoid tumor of soft parts, extraskeletal myxoid chondrosarcoma, and glial and chondroid choristomas or heterotopias. Although the histogenesis of this neoplasm is unclear, we suspect that a cell of undifferentiated ectomesenchyme is the progenitor and suggest the descriptive term ectomesenchymal chondromyxoid tumor (ECT) of the anterior tongue be adopted.

Adult↗

Quantitative assessment of basement membranes in soft tissue tumours. Computerized image analysis of laminin and type IV collagen.

Basement membranes (BMs) in 201 soft tissue tumours were quantified using computerized image analysis of tissues immunostained for laminin and type IV collagen. The purpose of the study was to compare and quantify the extent of BM deposition in a large and varied group of benign and malignant tumours. Laminin and type IV collagen gave similar results. The difference between benign and malignant was statistically highly significant (P = 0.0001), with greater deposition in benign tumours. BM deposition was homogeneous in benign tumours and heterogeneous in sarcomas and appeared to correlate with the degree of differentiation. Some poorly differentiated sarcomas showed cytoplasmic laminin staining but little or no extracellular BM. Immunohistochemical evaluation of BM has some advantages over electron microscopy; specialized equipment is not needed and since large samples can be studied with little sampling error, heterogeneity can be studied more readily. Subjective visual assessment gives a good overall indication of the extent of BM deposition and in many situations is likely to be a suitable alternative to image analysis. Because of staining heterogeneity, BM immunohistochemistry is unlikely to be of significant value in the diagnosis of specific types of sarcoma.

Basement Membrane↗

Desmoplastic small round cell tumors of the paratesticular region. A report of six cases.

Desmoplastic small round cell tumor (DSRCT) typically occurs in the abdomen but may also present at other sites. We report six cases of paratesticular DSRCT. The patients, who ranged in age from 17 to 37 (mean, 28) years, presented with a scrotal mass (five cases) or testicular pain (one case). Grossly, the tumors were white to tan and firm. Typically, they involved the paratesticular soft tissue, serosal surfaces and the epididymis near the junction with the rete testis. Microscopically, the tumors consisted of nests of mitotically active "small blue cells" with scant cytoplasm embedded in a densely fibrotic stroma. Two tumors showed focal tubule formation; one of these also formed rosettes. The tumors exhibited the typical immunophenotype of DSRCT (positivity for keratin, vimentin, desmin, and neuron-specific enolase but nonreactivity with HBA-71 and anti-S-100). Four tumors metastasized to lymph nodes (retroperitoneal, cervical, and two unspecified); pulmonary metastases occurred in one of these cases and in one patient without lymph node metastases. One of the above patients treated with chemotherapy, died of disease at 16 months. The patients with pulmonary metastases (one of whom also had lymph node metastases) were treated with aggressive chemotherapy and are alive and apparently disease-free at 2.5 and 3 years, respectively. Three of the six patients, two of whom had known metastases, were lost to follow-up. The DSRCT of the paratestis has histologic and immunohistochemical features identical to its abdominal counterpart and must be differentiated from other "small blue cell" tumors of the paratesticular region.

Adolescent↗