[Diagnosis and treatment of soft-tissue tumors]. / Prise en charge des tumeurs des parties molles de l'appareil locomoteur de l'adulte.

The diagnostic and therapeutic management of patients with soft-tissue tumors would be similar to the approach used for bone tumors if it were not for one crucial factor: the absolute necessity to recognize a sarcoma. The predominant features are the size of the tumor and its superficial or deep localization. If the tumor is small and superficial, biopsy can be associated with immediate resection without risk of dissemination to the deep tissues: this is the biopsy-resection approach. If the tumor is deep or superficial but large sized, search for locoregional spread with MRI is necessary before undertaking any surgical procedure. MRI can help guide the biopsy and plan resection if the tumor is a sarcoma. A first biopsy is necessary to establish the histological diagnosis and elaborate the therapeutic strategy. Samples should be sent immediately to the pathology lab which should examine sterile fresh tissue. Experience has demonstrated that proper rules for diagnosis and treatment are not necessarily applied initially in approximately one-fourth of all subjects with a malignant soft-tissue tumor. Besides the medical problems caused by this situation, the patient loses a chance for cure. When the tumor is a sarcoma, surgery is the basis of treatment. Complementary radiation therapy may be necessary, particularly for high-grade tumors or if the surgical margin was insufficient. Systemic or locoregional chemotherapy can also be used for high-grade or non-resectable tumors.

[Primary intraosseous rhabdomyosarcoma]. / Rhabdomyosarcome intra osseux primitif.

We report a case of primary intraosseous pleiomorphic rhabdomyosarcoma located in the pelvis of a 21-year-old woman followed for 4 years. The lytic tumor involved the acetabulum and the isthma with moderate extension to soft tissue. First line chemotherapy was unable to arrest tumor progression. Hemipelvectomy with saddle prosthesis reconstruction was performed, but septic complications dictated a secondary inter-ilio-abdominal amputation. Recurrence-free remission was achieved for 4 years, suggesting this was indeed a primary tumor. Primary intraosseous rhabdomyosarcomas are exceptional. Bone localizations generally suggest metastasis from a primary tumor often situated in an intraperitoneal localization. When search for a primary tumor is negative, intraosseous lesions can be considered as primary tumors warranting curative treatment. Radical surgical resection is recommended within the framework of a multidiscipinary management protocol associating radiotherapy and chemotherapy to improve prognosis.

[Osseous site of systemic cryptococcosis in an immunocompetent patient]. / Localisations osseuses d'une cryptococcose systemique chez un sujet immunocompétent.

Cryptococcosis is a cosmopolite mycosis caused by an opportunistic fungus usually infecting immunodeficient subjects. A 50-year old patient, with no particular medical history was seen with metaphyseal and diaphyseal bony lesions of a non-specific lytic aspect. This demonstrates that such an infection may also occur in apparently immunocompetent subjects. Diagnosis may be difficult and needs a special investigation looking for Cryptococcus neoformans. It should perhaps be made when first-intention investigations of a lytic bony lesion does not reveal any precise aetiology.

Taenia crassiceps invasive cysticercosis: a new human pathogen in acquired immunodeficiency syndrome?

A fluctuant, painful, subcutaneous, and intermuscular tumor developed in a 38-year-old man with severe acquired immunodeficiency syndrome (AIDS) in which immunodeficiency was severe. Surgery revealed lesions that formed a multilocular pouch embedded in deep tissues in the forearm filled with tapiocalike material containing a viscous fluid, granules, and cysticercilike small vesicles. Pathologic and parasitologic evaluation showed cysticerci embedded in a fibrocollagen reaction with inflammatory granulomatous reaction. Each cysticercus contained an invaginated scolex with two rows of small (i.e., 80 microm) and large (i.e., 114 microm) rostellar hooks, identical to larva of Taenia crassiceps. All clinical, parasitologic, and pathologic features of these cysticerci were very different from those of all other larval cestode (i.e., Taenia solium cysticercosis, coenurosis, sparganosis, cysticercosis due to Taenia saginata [Cysticercus bovis], primary and secondary hydatidosis [Echinococcus species]). T crassiceps cysticerci usually develop in subcutis and pleuroperitoneal cavities of rodents, whereas the adult tapeworm is commonly found in the digestive tract of foxes. Biologic properties of T crassiceps cysticerci and epidemiologic characteristics of pandemic human immunodeficiency virus (HIV) could eventually indicate new potential cases of T crassiceps cysticercosis in humans.

Spinal abscess and spondylitis due to actinomycosis.

STUDY DESIGN: Report of a rare case of spinal actinomycosis in a young immunocompetent woman. OBJECTIVE: To show the difficulties in diagnosing spinal actinomycosis. SUMMARY OF BACKGROUND DATA: Spinal actinomycosis is rare and usually occurs as a result of a contiguous (abdominal, pelvic, or thoracic) spread of the infection. This localization represents less than 5% of the infectious sites and was mainly, before the penicillin era, a postmortem discovery. METHODS: A case is reported of a 34-year-old Algerian woman who had fever, persistent cough, right-side thoracic pain, and progressive severe back pain. Radiographs, computed tomographic scan, and magnetic resonance imaging demonstrated lytic areas on the vertebral bodies of T11 and T12 and a paravertebral mass, without disk involvement. A surgical biopsy of T12 and the paravertebral abscess was performed. RESULTS: Presence of characteristic sulfur granules and gram-positive filamentous bacteria in surgical biopsy tissues and isolation of Actinobacillus actinomycetemcomitans in cultures led to the diagnosis of vertebral actinomycosis. The patient was virtually free of pain and fever after a 3-month regimen of ofloxacin and rifampicin (Rifadine, Marion-Merell, France) and was without recurrence after 18 months of follow-up. CONCLUSIONS: Actinomycosis of the spine, caused by the spread of a paraspinal abscess, is extremely rare. The previously poor prognosis has been transformed by antibiotics.

Molecular diagnosis of Ureaplasma urealyticum in an immunocompetent patient with destructive reactive polyarthritis.

Polymerase chain reaction (PCR) amplification, which is a useful method for detecting infectious agents in joints, has potential utility in the molecular diagnosis of venereal-associated arthritis. Among pathogens detected by this technique, Ureaplasma urealyticum, which is primarily associated with reactive arthritis (ReA), is also implicated in septic arthritis in immunocompromised patients. We report here a case of destructive polyarthritis, initially suggestive of septic arthritis, in an immunocompetent patient whose PCR positivity for U. urealyticum DNA in one joint, in conjunction with the disease outcome and histologic findings, led to the diagnosis of destructive ReA.

[Malignant transformation of multiple chondroma. Apropos of a case]. / Transformation maligne des lésions d'enchondromatose multiple. A propos d'un cas.

Malignant transformation of multiple chondromas observed in Ollier's disease is a well known possibility. In the clinical case of a 52 year old woman reported here, the sarcomatous transformation of two endochondromatous foci occurred successively in less than one year. One was located in the calcaneus, the other within the proximal metaphysis of the ipsilateral fibula. This case is worth reporting for two reasons: the successive occurrence of these sarcomatous lesions led to a amputation below the knee, later completed by an extended radical resection of the fibula. This combination allowed to preserve the knee with a good functional result and without local recurrence, this clinical case may be compared with that reported by Goodman in 1984. Both cases show that sarcomatous transformations may occur almost simultaneously after a long quiscent period and suggest the existence of a factor that could trigger or favor a malignant transformation process.

Leiomyosarcoma of the tibia. Report of a case.

A 48-year-old man with an unremarkable medical history was admitted for a painful swelling over the anteromedial aspect of his right leg. Radiographs disclosed heterogeneity of the proximal tibia, with increased uptake on the bone scan. Computed tomography findings consisted of heterogeneity of the proximal tibial metaphysis and diaphysis with subtle cortical osteolysis, periosteal appositions and soft tissue involvement. Magnetic resonance images showed low signal from the metaphysis, diaphysis and soft tissues on T1 sections that enhanced after gadolinium and converted to high signal on T2 images. Lung metastases were also found. Histologic features were consistent with leiomyosarcoma, which was considered to have originated in the tibia since no other primary localization was found. Combination chemotherapy was successful in eliminating the clinical manifestations and clearing the lung metastases. Six months later, the same chemotherapy regimen failed to improve a local and pulmonary recurrence and the patient died a few months later. Primary leiomyosarcoma of bone is a rare tumor, of which one of the most characteristic locations is the proximal third of the tibia. Magnetic resonance imaging with both T1- and T2-weighted sequences is essential to evaluate intramedullary and soft tissue tumor spread. To our knowledge, there are no characteristic signal patterns allowing to differentiate leiomyosarcoma from other primary malignancies of bone. Immunohistochemical and electron microscope studies are useful diagnostic tools.

[Clear cell sarcoma of the tendons and aponeuroses]. / Sarcome à cellules claires des tendons et aponévroses.

BACKGROUND: Clear cell sarcoma of tendons and aponeuroses is a rare mesenchymal tumor in childhood. It is difficult to treat and its prognosis is bad. CASE REPORT: A 9 year-old girl was admitted because of persistent pain in the left lower part of her thorax. Clinical examination showed a mass, 4 cm in diameter, embedded in the thoracic wall. Ultrasonography and CT-scan showed that this mass extended between the last ribs to the pleural cul-de-sac. There was no adenopathy and myelogram was normal. Biopsy of the mass showed features of clear cell sarcoma of tendons and aponeuroses. Cytogenetic studies showed translocation t(12;22) (q13-14;q12) in tumor cells. The patient was given chemotherapy followed by complete resection, but the tumor recurred locally 3 months later and the patient died, despite chemotherapy plus radiotherapy, 23 months after the apparent onset of disease. CONCLUSION: Treatment of this type of tumor remains difficult. The existence of a break point on 22q12, as in Ewing sarcoma, suggests that this tumor in of neural crest cell origin.