Amianthoid myofibroblastoma of the soft tissues

Myofibroblastoma (MF) is an uncommon, usually benign, mesenchymal tumor infrequently described in soft tissues. We report here on the clinicopathologic findings of a soft tissue MF (STMF) presenting in the neck of a 90-year-old man as a slowly growing and non-painful nodule, 4 cm in greatest diameter. Histology revealed a circumscribed lesion constituted of monomorphous bipolar spindle cells arranged in swirling fascicles with intervening broad bands of hyalinized collagen and well formed "amianthoid" fibers. Immunohistochemistry showed the spindle cells to be immunoreactive for vimentin, smooth muscle actin and muscle specific actin and, focally, for desmin; immunostaining for cytokeratin, epithelial membrane antigen, S-100 protein, factor VIII-related antigen, and CD34 was negative. Based on the present case and on those previously reported in the literature, STMF is characterized by: 1) exclusive incidence in the male sex; 2) variable immunoreactivity of the neoplastic cells for desmin, probably reflecting an origin from a peculiar subset of myofibroblasts, or, alternatively, a further myoid differentiation; 3) variable abundance of (hyalinized) collagen; 4) presence of amianthoid fibers. The combination of desmin immunoreactivity, frequently observed in MF of the breast, and amianthoid fibers, the main feature of MF of the lymph nodes, has never been observed in soft tissue MF. It is important to recognize STMF as a specific clinicopathologic entity to avoid confusion with other types of spindle cell proliferation and to differentiate it from other types of myofibromatosis.     

Ewing's sarcoma of the ribs. A report from the cooperative Ewing's sarcoma study

31 patients with primary Ewing's sarcoma of the ribs were treated according to the protocols of CESS 81, CESS 86P and CESS 86. The results of treatment were reviewed and analysed. 24 patients presented with localised disease and 7 with regional disease. 20 of 24 localised cases and 6 of 7 regional cases underwent tumour resection. All but 2 localised cases received irradiation. The cumulative relapse-free survival (RFS) rate of 31 patients was 61% at 12.8 years. Patients with poor prognosis had tumour of the upper ribs (P = 0.0338), the posterior component of the ribs (P = 0.0597), or regional disease (P = 0.0001). Tumour size, existence of pleural effusion, type of the surgical margin and response to chemotherapy were not significant prognostic factors. Most of the localised cases could be controlled by combined treatment, but in regional cases prognosis remained poor.     

Granulocytic sarcoma. A case with an unusual location

A 58-year-old male was admitted to our hospital with a preliminary diagnosis of bacterial endocarditis. After admission, echocardiography indicated the presence of vegetation-like tissues on both the left coronary cusp and the anterior mitral leaflet, although retrograde aortography two months earlier hadn't indicated an abnormal finding on the aortic cusp. The vegetation-like tissues had gradually enlarged despite the administration of antibiotics at another hospital, and aortic and mitral regurgitation had become severe. We decided to perform replacements of the aortic and mitral valves on the day of admission. During the operation, the aortic valve was found not to have vegetation, but an aneurysmal pouch on the left coronary cusp. It was supposed that either bacterial endocarditis or catheter injury had made a part of the aortic cusp weak and intolerant of diastolic pressure gradient, and as a result, the weakened part of the cusp progressively dilated into the left ventricle forming an aneurysmal pouch. To our knowledge, there have been only two previous reports of an aneurysmal pouch on the aortic cusp documented in the literature.     

Multifocal Ewing's sarcoma and hypercalcemia. A case report

An unusual case of Ewing's sarcoma in which the earliest sign of disease was the development of hypercalcemia is described. Radiologic examinations showed extensive osteolysis involving all of the trunk, and the proximal femora and humeri. An ill defined destruction of the left fibula was also noted. No definite primary site was found. The lungs and other visceral organs were free of metastasis. To the best of the author's knowledge, no comparable case has been reported in the literature.     

Intranodal palisaded myofibroblastoma: report of three new cases

Intranodal palisaded myofibroblastoma is a well-defined entity in the group of spindle-cell neoplasms of lymph nodes. The authors report 3 new cases of this unusual tumor. The nature of the proliferating cells is discussed.     

Kaposi's sarcoma pathogenesis: a link between immunology and tumor biology

Kaposi's sarcoma (KS) was a rare disease in Europe and North America until a decade ago, when it became the most common neoplasm complicating the acquired immunodeficiency syndrome (AIDS), where it acquires an aggressive course. Clinical and experimental data suggest that, at least in early stage, KS may not be a true sarcoma, but an hyperplastic-proliferative lesion that may regress. At least three components characterize KS lesions: (1) neoangiogenesis and proliferation of spindle-shaped cells of endothelial and macrophage cell origin, some of which may originate from a circulating precursor; (2) a cellular infiltrate represented by macrophages, lymphoid cells, mast cells, and neutrophils; and (3) the infection of spindle cells and mononuclear cells with a new virus of the Herpesvirinae family defined KS-associated herpesvirus or human herpesvirus-8 (HHV-8). KS lesions are highly responsive, in terms of growth, to inflammatory cytokines (IC) and many lesional cell components are able to secrete cytokines and chemokines, which induce paracrine-autocrine mechanisms of growth, angiogenesis, and promote further cellular recruitment. The association between HHV-8 and KS is close; however, the role of the virus in KS development is yet unknown. Nevertheless, the virus has the potential to encode for homologs of cellular cytokines and some chemokines and its reactivation is sensitive to stimuli provided by IC. This review focuses on these aspects of KS pathogenesis, trying to reconcile many of the clinical and experimental observations. Finally, the role of the HIV-1 Tat protein as a factor of progression in AIDS-KS as well as the role of cellular and HHV-8 encoded proto-oncogenes as factors and markers of progression of KS to a true malignancy is reviewed.     

Vitamin E protection against tumor formation by transplanted murine sarcoma cells

Mice fed vitamin E at a level of 0.5 g DL-alpha-tocopheryl acetate/kg diet demonstrated decreased incidence and rate of appearance of tumors produced by transplanted sarcoma cells (K3T3), compared to control groups fed diets without the vitamin supplement. Protection was dependent on the degree of unsaturation of dietary fat and on the size of the tumor cell challenge. When vitamin E was increased 10-fold (to 5 g/kg diet), the protective effect was no longer observed. Protection may be mediated through the host immune system, because sublethal, whole-body X-irradiation abrogated differences in tumor development between the +E and the -E mice. Studies with in vitro immunization showed that treatment of the K3T3 cell with vitamin E enhanced its ability to induce a cytotoxic response. It appears that the direct effect of vitamin E is on the tumor cell rather than on the immune system, since spleen cells from mice fed diets with and without vitamin E supplementation were indistinguishable in their response to untreated K3T3 cells. K3T3 cells treated with excessive levels of vitamin E were unable to induce a cytotoxic response, a result that correlates with the loss of protection against tumor development when massive doses of vitamin E were fed.     

Ewing sarcoma. Diagnostic imaging

Ewing's sarcoma is a highly malignant neoplasm of the bone whose origin is still uncertain. A strong relationship exists between Ewing's sarcoma and tumors of neural origin (Ewing family of tumors). Ewing's sarcoma must be distinguished from other round-cell tumors like lymphoma and neuroblastoma and also must be differentiated from osteogenic sarcomas. On plain radiographs, Ewing's sarcoma appears as a lytic or mixed lytic-sclerotic, rarely as predominantly sclerotic lesion with margins Lodwick grade III. It is located primarily in the diaphyseal and metadiaphyseal regions of the long bones of the lower extremities. A large soft tissue tumor is usually present. Magnetic resonance imaging is the imaging modality of choice to evaluate the extent of the primary lesion, to monitor the response to neoadjuvant chemotherapy and to follow up non-resected Ewing's sarcomas. Bone scintigraphy is necessary to detect skeletal metastasis, and 201thallium scanning has been shown to be sensitive in the monitoring of treatment response. Today, computed tomography is not longer used to image the tumor site; however, spiral CT of the lungs plays a central role as a staging and follow-up tool.     

Clear cell sarcoma of kidney with tumor extension into the right atrium

A 28-year-old man with a Wilms tumor extending into his right atrium was successfully treated using cardiopulmonary bypass. Histologic examination revealed clear cell sarcoma with invasion of the inferior vena cava. This case represents the oldest patient with clear cell sarcoma of the kidney ever published in the English literature.     

Frequent detection of tumor cells in hematopoietic grafts in neuroblastoma and Ewing's sarcoma

Many poor-risk neuroblastomas and tumours of the Ewing's sarcoma family (ET) recur despite autologous transplants. Recurrence may be due to tumor cells contained in the BM harvests or PBSC harvests. The objectives of this prospective study were to: (1) determine the incidence and degree of tumor cell contamination in paired BM and PBSC harvests; and (2) determine the efficacy of tumor cell purging by immunomagnetic CD34+ cell selection. 198 samples from 11 consecutive patients with neuroblastoma or Ewing's sarcoma were analyzed. We assayed tumor contamination by RT-PCR assay for PGP 9.5, plus immunohistochemistry for neuroblastoma-specific antigens (the latter in neuroblastoma only). None of these patients had tumor cells detected in their BM by clinical histology immediately before BM or PBSC harvests. However, 82% of PBSC and 89% of backup BM harvests were contaminated with tumor by RT-PCR and/or immunocytochemistry assays. Unselected PBSC and BM harvests contained similar quantities of tumor cells (median, approximately 200000 cells). Cyclophosphamide plus G-CSF mobilization did not affect the incidence or level of contamination in PBSC harvests, as compared to blood obtained before mobilization. Immunomagnetic CD34+ cell selection depleted tumor cells by a median of 3.0 logs for PBSC, and 2.6 logs for BM harvests.     

Embryonal sarcoma of the orbit. A clinical review of 19 Danish cases

The present study deals with 19 Danish cases of embryonal sarcoma of the orbit. Eight patients are still alive, however, only six with long-period survival (8-21 years). The contralateral orbit (and eye) remained uninvolved in the 19 cases; accordingly the six long-period survivors all have normal "social" vision. In 17 cases the homolateral eye was removed early or late in the clinical history. Only one of the long-period survivors has preserved the homolateral eye (and with useful vision, 6/24 after surgery for irradiation cataract). The nation-wide investigation covers a period of 22 years. It reflects the difficulties in diagnosing orbital tumours and the changes in therapeutic approach to date. Megavoltage radiotherapy plus chemotherapy immediately after the initial biopsy is now considered the therapy of choice; exenteration of the orbit is performed only in the case of a recurrence. Against the background of the present and an earlier study, centralization of Danish orbital malignancies in infants and children is advocated.     

Osteogenic sarcoma: noninvasive in vivo assessment of tumor necrosis with diffusion-weighted MR imaging

PURPOSE: To evaluate diffusion-weighted magnetic resonance (MR) imaging for detecting tumor necrosis in an animal model of osteogenic sarcoma. MATERIALS AND METHODS: Twelve rats with osteogenic sarcoma underwent T1-weighted unenhanced and gadolinium-enhanced spin-echo and diffusion-weighted spin-echo MR imaging. Histologic correlation was performed. Signal intensities, T2 relaxation times, normalized apparent diffusion coefficients, and relative signal intensity increases were calculated. RESULTS: On diffusion-weighted images, necrotic tumor showed low signal intensity (mean normalized apparent diffusion coefficient, 0.46 +/- 0.20 [1 standard deviation]), indicating rapid diffusion of water molecules as a result of loss of membrane integrity, while viable tumor showed high signal intensity (mean normalized apparent diffusion coefficient, 0.16 +/- 0.05; P < .0001). Differences in the T2 relaxation times and relative signal intensity increases between viable and necrotic tumor were not statistically significant. CONCLUSION: Normalized apparent diffusion coefficients are more accurate in differentiating between viable and necrotic tumor than are T2 relaxation times or relative signal intensity increases on contrast-enhanced images. Signal intensity overlap between viable and necrotic tumor on gadolinium-enhanced images may be caused by the small molecular size of the agent, which permeates the interstitial space freely, thereby also enhancing necrosis. Diffusion-weighted MR imaging depicts differences in diffusion and, ultimately, in membrane integrity between viable and necrotic tumor and may be used to monitor tumor viability during treatment.     

Basalioma terebrans of trunk skin. A neglected tumor

Small basal cell carcinomas of the trunk are usually regarded as relatively harmless. In contrast, large and ulcerating basal cell carcinomas may become a therapeutic challenge with a less favourable prognosis and high risk of recurrence. The development of mutilating giant basal cell carcinomas appears less due to the biological aggressive character of the tumors, but rather to the patients' attitude of neglect. The case of a 62-year-old patient, featuring such attitude, demonstrates how a common basal cell carcinoma of the trunk can grow over a period of years to a troublesome ulcerating tumor.     

Lipoblastoma. A rare, benign tumor in children

Lipoblastoma is a rare, benign tumour of embryonal fat seen almost exclusively in infancy and early childhood. It occurs mostly in the extremities, but it is also seen in other parts of the body. The tumour may grow rapidly, and the fact that lipoblastomas show immature fat cells could lead to the wrong diagnosis of liposarcoma. Complete surgical excision appears to be the treatment of choice. A correct, preoperative diagnosis is possible in most cases. Two cases of lipoblastoma of the upper limb and one case in the scapular region are reported.     

Primary central nervous system lymphoma. A changeable tumor

We have previously shown that lymphocyte beta 2-adrenoceptors (AR) are under cyclical control of sex-steroid hormones with greater receptor density during the luteal phase of the menstrual cycle. It has also been postulated that abnormal cyclical regulation of beta 2-AR might be a possible mechanism for premenstrual asthma. The effects of exogenous female sex-steroid hormones on lymphocyte beta 2-AR function were studied in eight normal healthy females. They were evaluated at two successive menstrual cycles, during the follicular phase (day 1-6). They were randomized to receive single oral doses of either ethinyloestradiol 50 micrograms or medroxyprogesterone 10 mg in a cross-over study. Lymphocyte beta 2-AR parameters were evaluated at baseline (t0), 24 h (t24) and 72 h (t72) after ingestion. Baseline levels of progesterone and oestradiol were comparable on both cycles. Receptor density (Bmax) increased significantly (P < 0.01) from t0 after progesterone but not oestradiol at t 4: a 1.39-fold geometric mean difference (95% CI 0.96-2.00) between t24 vs t0. Receptor affinity (kd) and maximal cAMP response to isoprenaline (Emax) were not altered by either treatment. These results show that exogenous progesterone but not oestradiol, given during the follicular phase, significantly increased beta 2-AR. This, therefore, suggests that endogenous progesterone is probably responsible for previously observed increase in Bmax during the luteal phase of the female menstrual cycle. These findings may suggest possible therapeutic strategies for modulation of beta 2-AR in premenstrual asthma.     

A paratesticular localization of desmoplastic small cell tumor. A case report

We report a case of paratesticular desmoplastic small cell tumor. In accord with the literature, the patient was subjected to treatment with combined chemotherapy, but he developed progression of disease and died 13 months later.     

Primary leiomyosarcoma. A rare tumor of the adrenal gland

OBJECTIVE: To report on a rare case of primary leiomyosarcoma of the adrenal. To our knowledge, this is the fourth case reported in the literature. METHODS: A patient with primary leiomyosarcoma of the adrenal gland is presented. The clinical features are described, and the diagnostic and therapeutic aspects of this rare primary mesenchymal tumor are discussed. RESULTS/CONCLUSIONS: The aggressive nature and the poor prognosis of this rare tumor type are emphasized.