Elastofibroma of the gluteal region with a concomitant contralateral lesion: case report and review of the literature.

A 51-year-old man presented with elastofibroma (EF) of the gluteal region with a concomitant contralateral lesion. The patient presented with a slow growing mass of the proximal third of the right buttock and had swelling, discomfort in sitting, and right-hip pain during walking for 2 months. On MRI, a soft-tissue mass was noted between the gluteus maximus and the gluteus medius muscle. The mass showed similar signal intensity to the surrounding tissue on T1- and T2-weighted images and with linear hyperintense areas in its internal structure. At surgery, a soft, non-encapsulated, irregular, and rubber-like mass was found attached to the gluteus medius muscles. It was pathologically confirmed to be an EF. This unusual manifestation of an EF is discussed.

Accuracy of water-enema multidetector computed tomography (WE-MDCT) in colon cancer staging: a prospective study.

PURPOSE: To assess the accuracy of water-enema multidetector computed tomography (WE-MDCT) in extra-rectal colon cancer staging. MATERIALS AND METHODS: Fifty-three patients (mean age 70 years) with extra-rectal colon cancer proven by colonoscopy and biopsy were prospectively evaluated by preoperative WE-MDCT. CT scans were both intraluminal (water enema or WE) and intravenous (iodinated) contrast enhanced (CE). All patients underwent surgery. Tumors were classified with the TNM staging system. Noted CT features were: tumor size and location; tumor form and edges; spread to the pericolic fat or neighboring organs; thickening of retroperitoneal fascia; number, size, and enhancement of the peritumoral lymph nodes. Tumors were classified on CT into 3 T-stage groups: T1/T2, T3, and T4. Lymph nodes were classified by their density after injection [positive over 100 Hounsfield units (HU)]. RESULTS: Tumor localization to the specific colon segment was correct in all the cases. The agreement between WE-MDCT staging and histopathology staging was good (k = 0.64). An irregular and bowl-shaped aspect of the external edges of tumor provided excellent sensitivity for T3/T4 inclusion (Se 97.7%, NPV 85.7%). Thickening of a fascia or the abdominal wall provided good specificity for T4 stage (Sp 88.1%, NPV 94.9%). Enhancement over 100 HU of at least one peritumoral lymph node was the best criterion of N+ staging (Sp 67.7%, NPV 87.5%). CONCLUSION: WE-MDCT permits good staging of colon cancer based on objective features.

Response to high-dose ifosfamide in patients with advanced/recurrent Ewing sarcoma.

AIM: To determine activity and toxicity of high-dose ifosfamide (HDIFO) in recurrent or advanced Ewing sarcoma family tumors (EFT). METHODS: Thirty-seven EFT patients [median age 17 years (6-45 years)] previously treated with chemotherapy regimens including standard dose ifosfamide were enrolled. HDIFO was administered for metastatic recurrent disease in 33 patients and for progression during neoadjuvant chemotherapy in 4 patients. All patients who received two courses of 15 g/m(2) ifosfamide were evaluable for radiographic response assessed according to RECIST criteria. RESULTS: Transient Grade 4 neutropenia and thrombocytopenia in 97% and 54% HDIFO courses respectively and severe CNS toxicity in one patient were observed. Thirty-five patients were evaluable: 12 (34%) had complete (2) or partial (10) response, 11 (32%) had stable disease, and 12 (34%) had progression. CONCLUSIONS: In patients with relapsed or advanced EFT previously treated with standard dose ifosfamide HDIFO is active and it should be considered a treatment option.

Parosteal osteosarcoma dedifferentiating into telangiectatic osteosarcoma: importance of lytic changes and fluid cavities at imaging.

PURPOSE: This study was performed to assess the imaging findings in cases of parosteal osteosarcoma dedifferentiated into telangiectatic osteosarcoma. Parosteal osteosarcoma is a low-grade well-differentiated malignant tumor. Dedifferentiation into a more aggressive lesion is frequent and usually visible on imaging as a central lytic area in a sclerotic mass. Only one case of differentiation into a telangiectatic osteosarcoma has been reported. As it has practical consequences, with a need for aggressive chemotherapy, we looked for this rather typical imaging pattern. MATERIALS AND METHODS: Review of 199 cases of surface osteosarcomas (including 86 parosteal, of which 23 were dedifferentiated) revealed lesions suggesting a possible telangiectatic osteosarcoma on imaging examinations in five cases (cavities with fluid). Histology confirmed three cases (the two other only had hematoma inside a dedifferentiated tumor). There were three males, aged 24, 28, and 32. They had radiographs and CT, and two an MR examination. RESULTS: Lesions involved the distal femur, proximal tibia, and proximal humerus. The parosteal osteosarcoma was a sclerotic, regular mass, attached to the cortex. A purely lytic mass, partially composed of fluid cavities was easily detected on CT and MR. It involved the medullary cavity twice, and remained outside the bone once. Histology confirmed the two components in each case. Two patients died of pulmonary metastases and one is alive. CONCLUSION: Knowledge of this highly suggestive pattern should help guide the initial biopsy to diagnose the two components of the tumor, and guide aggressive treatment.

Extra-axial chordoma: a clinicopathologic analysis of six cases.

Extra-axial chordoma is an exceedingly rare tumor, with only 28 cases reported in the literature to date. Axial and extra-axial chordoma exhibits complete morphologic and immunophenotypic (expression of brachyury) overlap. However, in consideration of the non-canonical presentation, extra-axial chordoma is under-recognized and often misdiagnosed, most often as extraskeletal myxoid chondrosarcoma or myoepithelioma. To increase our understanding of the clinicopathologic features of extra-axial chordoma, six cases have been retrieved from the files of the Istituto Ortopedico Rizzoli and of the General Hospital of Treviso. The clinicoradiologic, morphologic, and molecular features have been analyzed, and the follow-up was updated. Our series included four female and two male patients; their ages ranged from 20 to 67 years (mean 45.8 years). All patients presented with a single mass localized in four cases in the soft tissue (posterior arm, left leg, dorsal aspect of the foot, and popliteal fossa), and in two cases in the bone (radius and second metacarpal bone). Grossly, the neoplasm was lobulated, with a fleshy cut surface and a diameter ranging between 0.8 and 8 cm (mean 3.4 cm). Morphologically, all six cases showed an epithelioid cell proliferation organized in nests and cords demarcated by fibrous septa and set in an abundant extracellular myxoid matrix. Neoplastic cells featured hyperchromatic nuclei and abundant vacuolated cytoplasm. Immunohistochemically, all six cases were strongly positive for EMA, cytokeratin AE1/AE3, S100, and brachyury. INI1 nuclear expression was retained. Smooth muscle actin, calponin, p63, and GFAP were all negative. Fluorescent in situ hybridization (FISH) analysis did not reveal rearrangements involving NR4A3, FUS, and EWSR1 genes. At follow-up (mean 55 months), all patients were alive without disease after local surgical treatment. One patient underwent thigh amputation following multiple local recurrences and inguinal node metastases treated with marginal resection. In conclusion, primary extra-axial chordoma is an extremely rare neoplasm with distinct morphological and immunohistochemical features. Immunomorphology and molecular analysis allow distinction from both extraskeletal myxoid chondrosarcoma and myoepithelioma. Complete surgical resection appears to be curative.

Dedifferentiated chondrosarcoma with "adamantinoma-like" features: A case report and review of literature.

Dedifferentiated chondrosarcoma is defined by the presence of a low grade malignant cartilaginous component juxtaposed to a high grade malignant non-cartilaginous sarcomatous components. Only 4 cases in which the high grade component showed epithelial differentiation have been reported in the literature; three featured a squamous and the one a glandular epithelial component. Here we describe a case of dedifferentiated chondrosarcoma exhibiting epithelial "adamantinoma-like" basaloid features. The patient underwent wide resection of the proximal tibia and post-operative chemotherapy and died 8 months after the diagnosis due to lung and bone metastases.

Primary extraskeletal myxoid chondrosarcoma of bone: Report of three cases and review of the literature.

Extraskeletal myxoid chondrosarcoma is a rare neoplasm of soft tissue. The usual location is in deep parts of the proximal extremities and limb girdles in middle-aged adults. The bone location as primary location is extremely rare and few cases are reported. We present three cases arising in bone with molecular confirmation using both RT-PCR and FISH analysis. Patients include two men and one woman with an age of 62, 69 and 73 years old. The mean size of the lesion was 13cm (range 8-18cm). Tumors arose in the iliac bone in two cases and in the proximal humerus in the other case. At time of diagnosis the three cases show bone cortex and soft tissue involvement. On imaging, lesions have a lobular pattern, are purely lytic, but take up contrast medium after injection. Two patients are alive with disease (local recurrence and lung metastasis) after five years and five years and six months, respectively and one patient died of disease two years after the diagnosis. The primary extraskeletal myxoid chondrosarcoma of bone seems to have a more aggressive behavior than the soft tissue counterpart. The molecular confirmation of diagnosis using RT-PCR is necessary to do the differential diagnosis with other entities, in particular with myoepithelioma that shows similar morphological features and EWSR1 and FUS genes rearrangement.

Primary retroperitoneal soft tissue sarcoma: Imaging appearances, pitfalls and diagnostic algorithm.

Although retroperitoneal sarcomas are rare tumours, they can be encountered by a wide variety of clinicians as they can be incidental findings on imaging or present with non specific symptoms and signs. Surgical resection can offer hope of cure and patient outcomes are improved when patients are managed in high-volume specialist centers. Failure to recognize retroperitoneal sarcomas on imaging can lead to inappropriate management in inexperienced centers. Therefore it is critical that a diagnosis of retroperitoneal sarcoma should be considered in the differential diagnosis of a retroperitoneal mass with prompt referral to a soft tissue sarcoma unit. In particular, the most common retroperitoneal sarcoma subtypes, liposarcoma and leiomyosarcoma, have characteristic imaging appearances which are discussed. This review therefore aims to set the context and guide clinicians through a diagnostic pathway for retroperitoneal masses in adults which arise extrinsic to the solid abdominal viscera.

[Surgery of retroperitoneal sarcomas]. / Chirurgie des sarcomes rétropéritonéaux.

Retroperitoneal sarcomas comprise approximately 15% of all soft tissue sarcomas and 50% of all retroperitoneal tumours. Helical CT of the abdomen and pelvis, with selective use of MRI will establish the extent of the tumour, its retroperitoneal location, the degree of necrosis, and the evidence of metastasis. A CT guided core needle biopsy is the optimal pre operative tissue sampling. A complete surgical resection is the mainstay of the treatment with a rim of normal tissue that often requires removal of adjacent organs. The 5-year probability of local control is approximately 50%. The role of adjuvant therapy is evolving and at present should not be used outside the investigational setting.

[Function preserving surgery of limb sarcomas: history and improvements]. / Chirurgie des sarcomes des tissus mous des membres chez l'adulte : historique et avancées thérapeutiques.

In the seventies, limb-sparing surgery and radiation therapy appeared capable of successfully treating the great majority of adult patients with soft tissue sarcomas of the extremities. No survival benefit could be demonstrated in the patients selected for amputation. Microscopically positive surgical margins are related to a greater risk of local recurrence, whereas overall survival is compromised by high grade and large tumor size. Presurgery multidisciplinary discussion, management in reference center and management within cancer network independently predict conformity to the clinical practice guidelines. Reoperation should be a planned part of definitive management whenever the initial surgical procedure was done without a histologic diagnosis or was not planned to be a wide excision. Soft tissue flap reconstruction facilitates therapy for patients with locally advanced tumors so that patients with larger tumors can undergo resection, limiting complications and limb sacrifice. Vascular reconstruction is a feasible option in case of involvement of major vessels. Isolated limb perfusion (ILP) with TNF and chemotherapy is an efficient limb-sparing neoadjuvant therapy for locally advanced limb soft tissue sarcomas. Efficacy and safety of low-dose TNFalpha could greatly facilitate ILP procedures in the near future.

Evaluation of response after pre-operative radiotherapy in soft tissue sarcomas; the European Organisation for Research and Treatment of Cancer-Soft Tissue and Bone Sarcoma Group (EORTC-STBSG) and Imaging Group recommendations for radiological examination and reporting with an emphasis on magnetic resonance imaging.

At present, there is no standardised approach for the radiological evaluation of soft tissue sarcomas following radiotherapy (RT). This manuscript, produced by a European Organisation for Research and Treatment of Cancer-Soft Tissue and Bone Sarcoma Group (EORTC-STBSG) and Imaging Group endorsed task force, aims to propose standardisation of magnetic resonance imaging techniques and interpretation after neoadjuvant RT for routine use and within clinical trials.

Randomized trial of cytoreduction followed by intraperitoneal chemotherapy versus cytoreduction alone in patients with peritoneal sarcomatosis.

PURPOSE: In order to decrease loco-regional relapse after complete resection of peritoneal sarcomatosis (PS), the role of intraperitoneal chemotherapy (IPEC) was prospectively evaluated. METHODS: Patients (pts) with completely resected PS were randomized between adjunction of IPEC or not. IPEC consisted of doxorubicin, 0.1mg/kg and cisplatin, 15 mg/m(2) per day for 5 consecutive days. Primary endpoint was survival, measured as time from randomization to death. The scheduled number of patients needed was 40 in order to detect a minimal increase of 40% overall survival with the adjunction of IPEC with a power of 80%. RESULTS: Thirty-eight consecutive pts have been randomized, 19 in each group. Ratio of retroperitoneal (RPS) and visceral (VS) sarcomatosis were 9/10 and 6/13 in IPEC- and IPEC+ group, respectively. Histoprognostic grade, Sugarbaker's score and mean number of resected organs were similar in both groups. There were no toxic deaths and morbidity was similar in both groups (four pts in each group). The median follow-up is 60 months. The median local relapse-free, metastatic relapse-free survival and overall survival were identical in both groups (12.5, 18 and 29 months, respectively), with no difference between RPS and VS sarcomatosis. CONCLUSION: Administration of IPEC after a macroscopically complete surgery did not allow to increase greatly the outcome of pts. Complete surgery remains the cornerstone of the treatment of patients with sarcomatosis with best results for low grade sarcomatosis.

Normal and pathological breast, the histological basis.

Breast tissue is heterogeneous, associating connective and glandular structures, which grow and change cyclically under hormonal regulation. Hormones are also thought to be the main determinant of the major benign and malignant pathologies encountered in the breast. Benign lesions are more frequent and fibrocystic changes are by far the most common among them. They usually associate different entities, (adenosis, fibrosis, cysts and hyperplasia) but vary in intensity and extension. Thus, their clinical and radiographic presentation is extremely different from one patient to another. Adenofibroma is the most frequent tumour. It also undergoes modifications according to hormonal conditions. About 90% of malignant tumours are primary carcinoma. The incidence of intra-ductal carcinoma has risen dramatically since the development of screening because of its ability to induce calcification. Two mechanisms could be involved in the formation of calcification: one active (tumour cell secretion of vesicles), the other passive (necrotic cell fragments are released). Invasive carcinoma comprises numerous histological types. Stromal reactions essentially determines their shape: a fibrous reaction commonly found in ductal carcinoma creates a stellate lesion while other stroma, inflammatory (medullary carcinoma), vascular (papillary carcinoma) or mucinous determine nodular lesions whose borders push the surrounding tissue. The histological features which give rise to the radiographic pattern will be emphasised.

Breast radiological cases: training with BIRADS classification.

The American College of Radiology has established guidelines for outcomes monitoring known as the Breast Imaging Reporting and Data System. The last edition of the BIRADS classification includes mammography, but also ultrasonography and MRI. Radiologists must be used to the BIRADS lexicon and the BIRADS assessment, in order to clarify mammography reports and to facilitate communication with the other physicians. This work is a training on 20 mammography cases to be familiar with the BIRADS classification.

Use of radiology for the pathologist in the management of breast lesions.

Today radiology is an essential step in the pathological analysis of breast biopsies. It is determinant at each stage of the management of non palpable lesions, clusters of microcalcifications and opacities, whether this concerns the needle biopsy or the surgical excision. Firstly, an X-ray is necessary to ensure that the core needle biopsy specimen has been adequately sampled and when samples with microcalcifications are selected by the radiologist, management can be more specific and accurate. In the case of surgical specimens, the X-ray confirms the presence of the radiographic abnormality or the clip indicating the site of the surgical excision which guides sampling. Some radiographic features also provide information on underlying pathologies allowing management to be adapted accordingly. Radiographs are also important to ensure that microscopically detected microcalcifications or lesions exactly correspond to the radiographic abnormality in size and location. The paraffin block can also be X-rayed to select those containing microcalcifications for additional slicing. It is also important to identify the presence of modifications caused by the core needle biopsy (fibrosis, haemorrhage and inflammation) and to carefully recognize displacement of epithelial cells and pseudo-emboli resulting from the needle procedure. Such correlation between radiology and pathology is essential so that appropriate management of the specimen can be adapted and to avoid pitfalls arising from pre-operative procedures.

[Understanding CAD (computer-aided diagnosis) in mammography]. / Comprendre et utiliser le CAD (Aide informatisée au diagnostic) en mammographie.

Generalization of breast screening programs requires efficient double reading of mammograms, which allows reduction of false negative interpretations, but it may be difficult to achieve. CAD (Computed Aided Detection) systems are dramatically improving and can now assist in the detection of suspicious mammographic lesions, either suspicious microcalcifications, masses or architectural distortion. Characterization of the lesions is improving as well. CAD mammography might complete or substitute to "human" double reading. The aim of this review is to present the main CAD systems commercially available, review the principles of CAD and discuss the results of CAD mammography. Specifically, the role of CAD within breast screening program, according to the results of recent prospective studies will be discussed.

Spontaneous healing of an osteochondroma fracture.

Osteochondroma is the most common benign tumor of bone, usually asymptomatic. Fracture of an osteochondroma is a rare complication and has been recognized as a cause of pain. Treatment of this fracture is controversial and some authors suggest fracture as an indication for surgical excision. We present a case of fractured osteochondroma that healed without complication.

Metastasizing tenosynovial giant cell tumour, diffuse type/pigmented villonodular synovitis.

Tenosynovial giant cell tumour, diffuse type, also known under a variety of other terms including diffuse pigmented villonodular synovitis, tends to be locally aggressive and not infrequently can show multiple recurrences. The differential diagnosis with the extremely rare and somewhat controversial malignant variant of tenosynovial giant cell tumour, diffuse type, is challenging due to overlapping radiologic features of these two entities. Malignant tenosynovial giant cell tumour is defined by the presence of overtly malignant sarcomatous areas. We describe a very unusual case of a 63-year-old man affected by tenosynovial giant cell tumour, diffuse type of the knee that, despite absence of morphologic evidence of sarcomatous transformation, developed inguinal lymph node metastases following multiple surgical procedures.

Sclerosing epithelioid fibrosarcoma of the thigh: report of two cases with synchronous bone metastases.

We report two cases of sclerosing epithelioid fibrosarcoma occurring in the deep soft tissue of the thigh, confirmed by molecular analysis and associated with bone metastases in the lumbar vertebrae and the iliac wing at the time of diagnosis. Synchronous bone metastases of sclerosing epithelioid fibrosarcoma are extremely difficult to diagnose because clinical and radiological features are not specific. In addition, the range of differential diagnoses is very wide, including metastatic carcinoma and osteosarcoma. At present, all but three published cases of sclerosing epithelioid fibrosarcoma with bone metastases showed bone metastases during follow-up. We confirm in our two cases that the distinct pattern of immunohistochemical staining for MUC4, associated with the absence of staining for both SATB2, a marker of osteoblastic differentiation, and pan-cytokeratin, allows differentiating between sclerosing epithelioid fibrosarcoma and metastatic carcinoma or osteosarcoma.