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Pulmonary synovial sarcoma is a rare malignant tumor accounting for 10% of soft tissue sarcomas. It usually arises from the limbs and its occurrence in the lung is exceptional. We here report a clinical case of pulmonary synovial sarcoma in a 54-year-old man detected at a localised stage. This extremely rare tumor has a specific immunohistochemical phenotype. This provides strong support for diagnosis. Cytogenetic study confirms the diagnosis by showing the presence of specific translocation t (X; 18). This is highly characteristic of synovial sarcoma regardless of its anatomical location. This study highlights the anatomo-clinical, therapeutic and prognostic characteristics of this rare tumor often unknown to clinicians. [ABSTRACT FROM AUTHOR]

Résumé À travers une étude rétrospective portant sur 70 cas de tumeurs bénignes des parties molles de la main pris en charge au service d’orthopédie traumatologie de l’hôpital militaire de Tunis sur une période de 12 ans allant de janvier 2005 à décembre 2016, nous avons comparé nos résultats à ceux de la littérature pour essayer de dégager des notions épidémiologiques pertinentes pour la pratique médicale quotidienne vu que les données épidémiologiques concernant les tumeurs bénignes des parties molles de la main restent peu nombreuses et parfois discordantes. Les auteurs rapportent une série de 70 militaires actifs, jeune adultes, en majorité féminine, avec une tuméfaction au niveau de la main comme motif de consultation le plus courant. Le médius et l’index sont les doigts les plus touchés à fréquences égales. L’examen complémentaire clé est l’échographie des parties molles. Tous les patients ont été traités par une biopsie exérèse chirurgicale complète de leur tumeur suivie d’une étude histologique de la pièce opératoire. Trois cas de récidive ont été notés. À travers cette étude, on attire l’attention sur la fréquence importante des tumeurs des parties molles de la main dans une population particulière (agents militaires tunisiens). Par ailleurs, cette étude nous a permis de confirmer les données de la littérature concernant la dominance de la localisation digitale. En contre-partie, cette étude rapporte que la tumeur à cellules géantes est le type histologique le plus fréquent dans la population étudiée. Summary Through a retrospective study of 70 cases of benign soft tissue tumors in the orthopedic traumatology department of the military hospital of Tunis over a 12-year period from January 2005 to December 2016, we have compared our findings to those of the literature in an attempt to identify epidemiological notions relevant to daily medical practice as epidemiological data on benign tumors of the soft parts of the hand remain few and sometimes discordant. The authors report a series of 70 active, young adults, mostly female, with a swelling at the level of the hand as the most common reason for consultation. The middle finger and index finger were the most affected fingers at equal frequencies. The key supplementary examination was the ultrasound of the soft parts. All patients were treated with a complete surgical biopsy of their tumor followed by a histological study of the surgical specimen. Three cases of recidivism were noted. Through this study, the frequency of tumors of the soft parts of the hand in a particular population (Tunisian military agents) is emphasized. Moreover, this study allowed us to confirm the data of the literature concerning the dominance of the digital localization. On the other hand, this study reports that the giant cell tumor is the most common histological type in the study population. [ABSTRACT FROM AUTHOR]

Improvements in the treatment of soft tissue sarcomas should be achieved, particularly at the time of surgery, when the patient is curable. However, surgery is inadequate in more than half of the cases. It is fundamental to think about sarcoma before any surgery, follow the guidelines and refer the patients to a specialized center. Surgical results would be an important parameter of continuous improvement, which will be available through national databases. [ABSTRACT FROM AUTHOR]

Résumé: Les lymphomes non-hodgkiniens extraganglionnaires des tissus mous maxillofaciaux sont très rares. Nous rapportons deux cas de lymphome non-hodgkinien des parties molles maxillofaciales en rémission complète avec un recul de six et trois mois après une chimiothérapie suivie d’une radiothérapie localisée. Nous avons étudié les caractéristiques cliniques, radiologiques et histopathologiques ainsi que le traitement et le pronostic des lymphomes non-hodgkiniens extraganglionnaire des muscles maxillofaciaux. [ABSTRACT FROM AUTHOR]

Abstract: Myoepithelial soft tissue tumors represent less than 1% of soft tissue tumors. Diagnosis is based on histology, but clinical and radiological features can orient the practitioner. Myoepithelial tumors do not involve the bone as they are not malignant. In this article, we report a case of a myoepithelioma occuring on the volar aspect of the right ring finger involving the first phalanx. [Copyright &y& Elsevier]

Abstract: Solitary fibrous tumour is unusual, arising most commonly in the pleura and can also occur in a large number of other sites. We report the case of a 34-year-old man with a retroperitoneal solitary-fibrous tumour, revealed by abdominal pain and hypoglycaemia. We describe the histopathological and immunohistochemical features. Solitary-fibrous tumour should be included in the differential diagnosis of spindle cell tumours in this location. Despite complete local excision, local recurrence and metastasis are seen. The behaviour of theses tumours is unpredictable and patients with solitary fibrous tumour require careful and long-term follow-up. [Copyright &y& Elsevier]

The main role of the radiologist is to suggest a possible sarcoma in front of a soft tissue mass, when it is large (more than 3 cm) or in a deep location or pediatric, and to refer the patient to a well trained team. MRI is the best imaging technique for staging and follow-up. [ABSTRACT FROM AUTHOR]

Abstract: Gastrointestinal stromal tumors (GIST) are mesenchymal tumors that arise from the wall of the gastrointestinal tract expressing CD117 and/or the CD34. Similar tumors were described in the soft tissue of the abdomen and are so-called extragastrointestinal stromal tumors (EGIST). We report 4 new cases of EGIST. The tumors occurred in 2 women and 2 men, who ranged in age from 42 to 71 years. Three tumors arose from the soft tissue of the abdominal cavity, and the remainder arose from the retroperitoneum. They ranged in size from 10 to 27 cm. Three cases were composed purely of short fusiform cells tumors, the last case showed an epithelioid pattern. All the tumors expressed CD117. One patient presented with local recurrence and metastasis, one was lost to the follow-up and two patients were doing well. Immunohistochemistry is useful in distinguishing EGIST from other mesenchymal tumors. Intra-abdominal aggressive fibromatosis may express actin and CD117 but catenin is also positive, leiomyosarcoma expresses the actin and/or desmin but CD117 is usually negative, retroperitoneal dedifferentiated liposarcoma is CD117 negative and PS100 positive, inflammatory myofibroblastic tumor is negative for CD117 and CD34. The solitary fibrous tumor expresses CD34 and is negative to CD117. Some tumors expressing CD117 such desmoplastic round cell tumor should not be confused with EGIST. On the occasion of these four observations, we will discuss the clinical aspects and the main differential diagnoses of this tumor. [Copyright &y& Elsevier]

Abstract: The primitive neuroectodermal tumours (PNET) of soft tissues belong to the Ewing''s tumors family and affects particularly the child. The localization of the disease at the extremities is very rare within the adult population and raises the problem of differential diagnosis with others tumors of the soft tissues. We report the case of a 48-year-old patient with a localized tumor, at the level of the second right finger, of six months evolution. The biopsy showed the infiltrating nature of the tumour; and the diagnosis of (PNET) was confirmed after the histological and immunohistochemical study. The extension assessment was negative and the patient had an amputation of the second and third rays of the right hand. Four years afterwards, the patient showed no recurrence or metastases. [Copyright &y& Elsevier]

Abstract: Giant cell tumour of tendon sheath is a benign proliferative lesion of synovial origin that may affect the joints, bursae and tendon sheaths. It is the second most common soft tissue tumor of the hand after ganglion cyst. The localised (nodular) form is the most common. However, the less-common diffuse-type giant cell tumour is usually located in the peri-articular soft tissue. The authors report the case of a giant cell tumor of the tendon sheath arising from the carpal tunnel of the wrist in a 42-year-old woman. The patient presented a mild carpal tunnel syndrome and a mid-palmar swelling. We present an unusual localization of giant cell tumor of the tendon sheath, causing carpal tunnel syndrome. [Copyright &y& Elsevier]

Résumé: Nous décrivons les signes cliniques, radiologiques et histologiques d’un chondrome extraosseux dont la particularité consistait en une calcification diffuse de la matrice chondroïde. Le bilan a comporté une tomodensitométrie et une imagerie par résonance magnétique. Les images pondérées en T1 ont mis en évidence une masse bien limitée, en hyposignal, située dans le fléchisseur profond des doigts gauche et entourée d’un discret œdème intramusculaire. L’examen histologique a révélé un chondrome des parties molles fortement calcifié et responsable d’une réaction macrophagique à corps étranger. Une bonne connaissance de cet aspect inhabituel de calcification diffuse peut faciliter le diagnostic radiologique du chondrome extraosseux. [Copyright &y& Elsevier]

A case of intraosseous Ewing sarcoma is reported. This pathology of the young adult is very rare, clinical or imaging (CT or MRI) findings are non-specific and diagnosis is based on histology. Nonethless, this diagnosis should be considered in all patients with primary soft tissue tumors. [ABSTRACT FROM AUTHOR]