[Sarcoma related genes in bone soft tissues].
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Bone Neoplasms↗
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[Histopathological studies of temporal bones of patients with malignant melanoma].
Although malignant melanoma is known to metastasize to various sites including the temporal bones, there have been few studies on temporal bone histopathology in malignant melanoma. Here we describe the temporal bone histopathology of 5 patients (10 temporal bones) who died of malignant melanoma with multiple metastasis to many internal organs and bones. We investigated the temporal bone based on the following three points: 1) the presence of metastatic lesions in the temporal bone, 2) inner ear pathology, and 3) the distribution of melanin in the inner ear. Normal melanin distribution was also studied in 35 temporal bones of patients without malignant melanoma. Metastatic malignant melanoma was observed in 5 temporal bones from 3 patients, in two of whom the internal auditory canal was involved bilaterally by melanoma cell infiltration. In the remaining patient (one ear), metastatic melanoma was found along the dura mater of the posterior cranial fossa to the mastoid air cells. In the former two patients, the inner and outer hair cells as well as the stria vascularis showed degenerative changes to various extents. In particular, the inner ear changes were severe in the ear with the decongestion of the inner ear vessels. Melanin was found in the modiolus, stria vascularis, osseous spiral lamina, membranous labyrinth, and endolymphatic sac, as previously reported. The amount of melanin in the inner ear increased with age in the control patients, but was greater than in the controls, in all of the cases of malignant melanoma except one, in which metastatic lesions were present in the internal auditory canal with marked congestion of the inner ear vessels.
Adult↗
Postradiation sarcomas of the pelvis after treatment for uterine cervical cancer: review of the CT and MR findings of five cases.
OBJECTIVE: To characterize the radiologic features of postradiation sarcomas arising in the pelvic bones following treatment for uterine cervical carcinoma. DESIGN AND PATIENTS: Five patients who developed postradiation sarcomas in the pelvic bones following radiation therapy for carcinoma of the uterine cervix within the irradiated field were evaluated. Pelvic radiographs, computed tomography (CT) and magnetic resonance (MR) imaging were undertaken in all patients. Histologic confirmation of the tumor type was obtained. RESULTS: Three patients whose tumors were characterized as an osteosarcoma, an angiosarcoma and a malignant fibrous histiocytoma (MFH) showed a large round or oval mass mainly in the sacroiliac joint which extended into the posterior gluteal soft tissues. In a fourth patient an osteosarcoma developed in the central ilium extending widely into the soft tissues both anteriorly and posteriorly, with calcified areas within the extraosseous mass. The fifth patient had a MFH which showed osteolytic destruction of the cortex of the acetabulum, and minimal soft tissue extension. There were no specific features or signal intensity changes on MR imaging to differentiate these cases from primary sarcomas. CONCLUSION: Postradiation sarcoma must be considered in patients with uterine carcinoma when a soft tissue mass is seen in the previously irradiated field, especially if the mass is posterior to the sacroiliac joint and the latent period is more than 5 years.
Aged↗
[Malignant tumors of the head and neck in children. Part I--review].
About 27% of malignant tumors in children is located in the head and neck. Besides primary solid tumors, lymph node metastases as well as signs of systemic diseases of the lymphatic tissue are often found in this region. The latter are the most common childhood malignancies. Among primary solid malignant tumors in this region neoplasms of the soft tissues are frequency found. Their prevalence comes next to the tumors of the central nervous system and the orbit. Rhabdomyosarcoma (RMS) represents 50% of cases in this group. Malignant neoplasms originating from epithelial and bone tissue occur rarely in children. The evolution of triple therapy, combining surgery with irradiation and chemotherapy has produced and improvement in prolonged survival rates in these children.
Child↗
Management of extraosseous ewing sarcoma of the infratemporal fossa.
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Adolescent↗
Metastatic acinic cell carcinoma in a neurofibroma mistaken for carcinosarcoma.
BACKGROUND: Tumor-to-tumor metastasis is a rare, but well-recognized, entity most commonly involving metastatic carcinoma to a mesenchymal neoplasm. We report a case of acinic cell carcinoma of the parotid gland metastatic to a neurofibroma. METHODS AND RESULTS: A 55-year-old man with a history of a high-grade acinic cell carcinoma of the parotid was seen with a mass at the surgical site and metastatic foci in the scalp 10 months postoperatively. The resection specimen revealed a spindle cell lesion with metastatic foci of high-grade adenocarcinoma, initially diagnosed as a carcinosarcoma. The bland morphology and S-100-positive expression of the spindle cell lesion confirmed the diagnosis of neurofibroma. The high-grade features of the carcinomatous foci and their similarity to the primary tumor confirmed the presence of a tumor-to-tumor metastasis. CONCLUSION: To our knowledge, this is the first reported case of acinic cell carcinoma metastatic to a neurofibroma, an important entity in the differential diagnosis of biphasic tumors of the head and neck.
Carcinoma, Acinar Cell↗
Cancer incidence and mortality among teenagers in Vaud, Switzerland, 1974-1992.
Data from the Vaud Cancer Registry, Switzerland, were used to analyse incidence and mortality from cancer in teenagers (aged 10 to 19 years) over the period 1974-1992. A total of 113 males and 87 females were registered. Of these, 23% were lymphomas, 16% leukaemias, about 15% central nervous system neoplasms, 10% germ cell tumours and bone neoplasms and 8% soft tissue sarcomas. The overall incidence rate (age-adjusted, world standard population) for all cancers combined was 167 per million boys and 128 per million girls. In both sexes, there was some indication of rising trends over time, to reach 196 per million males and 141 per million females in 1986-1992. The only types of cancer showing consistent upward trends in both sexes were lymphomas. A total of 53 cancer deaths were certified, due to leukaemias in about 40% of cases in both sexes and to lymphomas and brain tumours in 15%-20%. The overall mortality rate at age 10-19 years (age-standardised, world standard) was 47 per million boys and 31 per million girls, and no trend in mortality was observed over time. The 2 main findings of our analysis are (i) the absence of major trends in cancer incidence in adolescents, with the sole exception of a possible increasing incidence of lymphomas, and (ii) the lack of appreciable trends in mortality, in view of the declines in cancer mortality observed in children and young adults over the same calendar period.
Adolescent↗
Cancer incidence among adolescents in France.
BACKGROUND: In France, cancer ranks third as the most significant cause of mortality in young people. However, the incidence, the survival, and the management of adolescent cancers have never been studied. The aim of this study is to investigate incidence rate (IR) of adolescents with cancer from data recorded in French Cancer Registries covering eight administrative areas, representing 10% of the French population, over a 10-year period (from 1988 to 1997). PROCEDURE: Data from the FRANCIM network of French population-based Cancer Registries were used to analyze cancer incidence among adolescents aged from 15 to 19 years, excluding basal cell carcinomas of skin. RESULTS: In total, 699 cases were recorded. Of these, 22.9% were lymphomas, 12.7% germ-cell tumors, 11.9% leukemias, 10.6% central-nervous-system tumors, 10.0% bone neoplasms, 7.6% soft-tissue sarcomas, and 19.5% tumors of adulthood (thyroid carcinomas 4.9%, melanomas 9.0%, and other carcinomas 5.6%). The overall IR was 172.9 per million adolescents (M/F: 1.2) with an annual increase of 3% (P = 0.58). Over the two 5-year periods (1988-1992 and 1993-1997) the IR increases significantly for malignant melanomas (respectively 10.4 and 21.2; P = 0.04). CONCLUSIONS: Our findings are similar to that reported by previous studies performed in European and North-American countries. Future studies need to focus on the etiology explaining the increase in incidence, the management and the impact of the type of care on outcomes.
Adolescent↗
Case report 751: Spindle cell lipoma causing marked bone erosion.
We present an unusual case of spindle cell lipoma of the foot which produced marked bone erosion. Spindle cell lipoma was initially described in the mid-1970s as having the characteristics of a lipoma with partial or total replacement of mature fat by collagen-forming spindle cells. This lesion is most commonly found in the subcutaneous tissue of the posterior neck and shoulder in men between 45 and 65 years of age.
Aged↗
Spectroscopic imaging of bone marrow composition in vertebral bodies.
The proton spectroscopic imaging technique that uses read gradient during acquisition was used for the measurement of the proton spectra in the lumbar and thoracic part of the spine of a patient with breast cancer without known skeletal metastases. The bone marrow fat/water ratios were evaluated in the same location before and after chemotherapy treatment. The results were corrected for relaxation effects. The fat/water ratios showed a significant increase as a consequence of the bone marrow degradation process due to chemotherapy. The proposed spectroscopic imaging technique offers rapid acquisition of proton spectra from large volumes of the vertebral bodies.
Adipose Tissue↗
MRI of bone metastases.
Some knowledge of MR theory is required to be able to achieve high contrast between bone metastases and normal marrow. Three factors are used in MR to diagnose bone metastases; fat-water distribution, artifacts induced by bone trabeculae, and uptake of contrast medium. Using MR-histological correlations based on specimens of the lumbar spine, and studies of patients, we explain the advantages and limitations of sequences studying fat and water (spin-echo T1, STIR, in- and out-of-phase gradient echo, fat presaturation), bone trabeculae (gradient echo with long TE), and the injection of contrast medium.
Adipose Tissue↗
Maxillofacial manifestations of multiple myeloma. An unusual case and review of the literature.
Oral symptoms and signs of multiple myeloma are common. An unusual case with pathologic fracture of the pterygoid process and external root resorption is presented. The literature is reviewed with respect to the prevalence of maxillofacial manifestations of multiple myeloma and the presence of such manifestations as the first sign and/or symptom of disease.
Female↗
The role of transsphenoidal microsurgery in the management of sellar and parasellar meningioma.
We report on eight meningiomas that have been operated on via the transsphenoidal route since May 1988. Six patients harbored cavernous sinus meningiomas with intrasellar extension. The intrasellar tumor extension and the parasellar tumor portion medial to the carotid artery were removed. Decompression of the pituitary gland with normalization of prolactin levels was achieved in all patients. It is a well-tolerated approach to confirm the diagnosis and still allows the option of major transcranial surgery in the event of tumor progression. Additionally, we report complete tumor removal in two rare cases with intrasellar and suprasellar merely subdiaphragmatic meningiomas.
Adult↗
Low-grade myofibroblastic sarcoma of the distal phalanx.
Low-grade myofibroblastic sarcoma is a recently defined tumor that is part of the spectrum of malignant mesenchymal tumors, showing features of myofibroblastic differentiation. These tumors occur most commonly in the head and neck regions, especially in the tongue, and less frequently in the limbs, trunk, and abdominal/pelvic cavity. We describe a 51-year-old woman with a low-grade myofibroblastic sarcoma. The distal phalanx of the finger was amputated with a clear surgical margin. The treatment of this tumor should include a simple local excision with free surgical margins and a long clinical follow-up period with imaging studies. At the 28-month follow-up evaluation the patient was free from this tumor.
Amputation, Surgical↗
Chondromyxoid fibroma involving the entire metacarpal: a case report.
The occurrence of chondromyxoid fibroma in the hand is rare. We report a case of chondromyxoid fibroma involving the whole fourth metacarpal that was treated by curettage and cancellous bone allograft.
Adult↗
Histogenesis of unique elastinophilic fibers of elastofibroma: ultrastructural and immunohistochemical studies.
Electron microscopy and both light and electron microscopic immunohistochemical tests for elastin were employed to study the morphogenesis of the unique elastinophilic fibers of an elastofibroma removed from the subscapular region of a 62-year-old woman. Ultrastructurally, as shown by tannic acid stain, elastinophilic fibers of the elastofibroma consisted of central cores and outer zones. The latter were composed of various sizes of vaguely demarcated, irregularly shaped amorphous components and compactly and randomly arranged large amounts of microfibrils. The electron microscopic immunohistochemical results showed that the small-sized amorphous components and microfibrils in the outer zones of the elastinophilic fibers were stained evenly and of granular texture, but the vaguely outlined large amorphous components were not stained. These findings were interpreted as indicating that the amorphous components of the outer zones of elastinophilic fibers were less compact and allowed the penetration of antielastin antibody. The unique elastinophilic fibers of elastofibromas appear not to be formed by the degeneration of the fibers but by abnormal elastogenesis, including an abnormal arrangement of microfibrils.
Bone Neoplasms↗
Hyperostotic macrodactyly and lipofibromatous hamartoma of the median nerve associated with carpal tunnel syndrome.
A new case with 14-year follow-up of an extremely rare variety of congenital hand macrodactyly is presented. The disease characteristically presents a diffuse proliferation of fibrofatty tissue, but in this special type, osteocartilaginous deposits around the joints can also be found. The case presented included the troublesome feature of a lipofibromatous hamartoma in the median nerve at the wrist and its branches producing carpal tunnel syndrome. The patient obtained benefit from carpal tunnel release and epineurolysis. The hyperostotic development was managed with conservative resection of the periarticular osteochondromas. The literature reviewed suggests that the hyperostotic cases of macrodactyly do not differ from general cases of this congenital condition, except for the osteochondral deposits. These tumours develop during adulthood or after previous trauma, before epiphyseal closure.
Adipose Tissue↗
MR imaging of the forefoot: Morton neuroma and differential diagnoses.
Magnetic resonance (MR) imaging of Morton neuromas is highly accurate. Morton neuromas are more conspicuous when the patient is prone positioned and the foot is plantar flexed than in the supine position with the toes pointing upward. MR imaging of Morton neuromas has a large influence on the diagnostic thinking and treatment plan of orthopedic foot surgeons. The most common differential diagnoses include intermetatarsal bursitis, stress fractures, and stress reactions. Some diagnoses (nodules associated with rheumatoid arthritis, synovial cyst, soft tissue chondroma, and plantar fibromatosis) are rare and can be diagnosed with histologic correlation only.
Bursitis↗