Somatic Soft Tissue Research Articles

Pancreatic involvement by mesenchymal chondrosarcoma harboring the HEY1-NCOA2 gene fusion

Mesenchymal chondrosarcoma (MC) is an aggressive small, round, blue cell tumor with chondrogenic differentiation that typically arises in bony sites. Approximately, a third of these tumors develop in extraskeletal sites such as the meninges, and somatic soft tissue. The MCs are well-circumscribed, lobulated masses, with focal calcification. Histologically, 2 distinct populations of neoplastic cells characterize MC: sheets of primitive small, round, blue cells surrounding islands of well-developed hyaline cartilage with mature chondrocytes in lacunae. Involvement of the gastrointestinal tract and pancreas by primary or metastatic MC is a relatively rare occurrence. We identified 8 patients with MC in our departmental archives from 1990 to 2015, two of which had pancreatic involvement. The patients were young women who developed masses in the distal pancreas. Molecular testing demonstrated that both tumors harbored the recently described HEY1-NCOA2 gene fusion. These cases illustrate that pancreatic involvement can occur in MC, and the demonstration of HEY1-NCOA2 fusion can be helpful to confirm the diagnosis.

Recurrent BCOR Internal Tandem Duplication and YWHAE-NUTM2B Fusions in Soft Tissue Undifferentiated Round Cell Sarcoma of Infancy: Overlapping Genetic Features With Clear Cell Sarcoma of Kidney.

Soft tissue undifferentiated round cell sarcoma (URCS) occurring in infants is a heterogenous group of tumors, often lacking known genetic abnormalities. On the basis of a t(10;17;14) karyotype in a pelvic URCS of a 4-month-old boy showing similar breakpoints with clear cell sarcoma of kidney (CCSK), we have investigated the possibility of shared genetic abnormalities in CCSK and soft tissue URCS. Most CCSKs are characterized by BCOR exon 16 internal tandem duplications (ITDs), whereas a smaller subset shows YWHAE-NUTM2B/E fusions. Because of overlapping clinicopathologic features, we have also investigated these genetic alterations in the so-called primitive myxoid mesenchymal tumor of infancy (PMMTI). Among the 22 infantile URCSs and 7 PMMTIs selected, RNA sequencing was performed in 5 and 2 cases, with frozen tissue, respectively. The remaining cases with archival material were tested for YWHAE-NUTM2B/E by fluorescence in situ hybridization (FISH) or reverse transcription-polymerase chain reaction (RT-PCR), and BCOR ITD by PCR. A control group of 4 CCSKs and 14 URCSs in older children or adults without known gene fusion and 20 other sarcomas with similar histomorphology or age at presentation were also tested. A YWHAE-NUTM2B fusion was confirmed in the index case by FISH and RT-PCR, whereas BCOR ITD was lacking. An identical YWHAE-NUTM2B fusion was found in another URCS case of a 5-month-old girl with a back lesion. The remaining cases and control group lacked YWHAE gene rearrangements; instead, consistent BCOR ITDs, similar to CCSK, were found in 15/29 (52%) infantile sarcoma cases (9/22 infantile URCS and 6/7 PMMTI). In the control cohort, BCOR ITD was found only in 3 CCSK cases but not in the other sarcomas. Histologically, URCS with both genotypes and PMMTI shared significant histologic overlap, with uniform small blue round cells with fine chromatin and indistinct nucleoli. A prominent capillary network similar to CCSK, rosette structures, and varying degree of myxoid change were occasionally seen. BCOR ITD-positive tumors occurred preferentially in the somatic soft tissue of the trunk, abdomen, and head and neck, sparing the extremities. RNAseq showed high BCOR mRNA levels in BCOR ITD-positive cases, compared with other URCSs. In summary, we report recurrent BCOR exon 16 ITD and YWHAE-NUTM2B fusions in half of infantile soft tissue URCS and most PMMTI cases, but not in other pediatric sarcomas. These findings suggest a significant overlap between infantile URCS and CCSK, such as age at presentation, histologic features, and genetic signature, thus raising the possibility of a soft tissue counterpart to CCSK.

Potential syndromes linking dispersal and reproduction in the hermaphrodite land snail Cornu aspersum

AbstractDispersal plays a key role in many ecological and evolutionary processes, in particular through correlations, or syndromes, with other life‐history traits. Here, we investigated the potential syndromes linking movement behaviour, body mass and male and female sexual organs development, to explain a previously described subadult dispersal pattern in the hermaphrodite land snail Cornu aspersum. We found elements indicating that this snail may not strictly be a simultaneous hermaphrodite, but presents a male‐biased phase before reaching adulthood and hermaphroditism. Body mass was positively correlated with both patch‐leaving propensity and movement speed. However, because the dry mass of the female albumen gland, which represents on average in adults masses equivalent to 42.6% of somatic soft tissues dry mass, was negatively correlated with patch‐leaving propensity, snails of intermediate age are expected to be more likely to leave than older ones. No relationship between male organ size and movement characteristics was found. We discuss briefly the interesting consequences the existence of a syndrome linking movement propensity with female investment in a protandric hermaphrodite species might have in terms of life‐history evolution and on the maintenance of hermaphroditism in dispersal‐favouring environments.

A mitotically active somatic soft tissue tumour of uncertain potential in a 16 year old male

A mitotically active somatic soft tissue tumour of uncertain potential in a 16 year old male

Epithelioid rhabdomyosarcoma: a clinicopathological study of seven additional cases supporting a distinctive variant with aggressive biological behaviour

We present our experience with seven cases of epithelioid rhabdomyosarcoma (RMS) to further characterise its clinicopathological features. There were five males and two females with ages ranging from 19 to 84 years (mean 56 years). Four tumours occurred in the somatic soft tissue, two in organs and one in the bone. The mean tumour size was 10.7 cm (range 3.5-15 cm). Histologically, six tumours were characterised by sheet-like growth of uniform epithelioid cells with large vesicular nuclei, prominent nucleoli, high mitotic activity and moderate to abundant amphophilic-to-eosinophilic cytoplasm. One tumour was composed of dyscohesive cells with rhabdoid appearance embedded in a myxoid matrix. Features suggestive of rhabdomyoblastic differentiation were absent. However, immunohistochemical study revealed skeletal muscle differentiation in all cases. Of note, focal expression of epithelial markers with co-expression of neuroendocrine markers was noted in five and three cases, respectively. Of six patients with follow-up, one experienced local recurrence and three developed metastases. To date, three patients have died of disease within 14 months. This study further demonstrates that epithelioid RMS represents a distinct variant of RMS with an aggressive behaviour. It may be misdiagnosed as poorly differentiated neuroendocrine carcinoma due to co-expression of epithelial and neuroendocrine markers.

Cutaneous PEComa

PEComa is a mesenchymal neoplasm thought to derive from the perivascular epithelioid cell, a hypothetical cell type not yet identified. PEComa is a broad term that was recently expanded to include visceral, retroperitoneal, and somatic soft tissue, in addition to cutaneous tumors, which share morphological and immunohistochemical features in common with angiomyolipoma, lymphangiomyomatosis, and clear cell "sugar" tumor. The latter have distinct, site-related, clinical, morphological, and biological features. PEComas share features of concurrent melanocytic and myocytic differentiation. Most cases are sporadic, but renal and pulmonary PEComas can be part of the tuberous sclerosis syndrome more often than PEComas from other sites. We report a case of cutaneous PEComa to raise awareness of the entity occurring at this site and caution against misinterpretation as a primary or metastatic malignancy, for example melanoma or renal cell carcinoma, clear cell type.

Epithelioid Leiomyosarcoma Arising in the Subcutis—A New Case Report of a Rare Variant

Epithelioid Leiomyosarcoma Arising in the Subcutis—A New Case Report of a Rare Variant

Myxoid Variant of So-called Angiomatoid “Malignant Fibrous Histiocytoma”

Angiomatoid "malignant fibrous histiocytoma" (AMFH) is a tumor of intermediate malignancy and undefined lineage, mostly arising in the extremities of young patients. Examples with a prominent myxoid matrix are very uncommon. Twenty-one cases of myxoid AMFH (among a total of 414) identified in consult files are described, including clinicopathologic features, fluorescence in situ hybridization analysis in a subset of cases, and follow-up. Thirteen patients were female and 8 male, ranging in age from 2 to 51 y (median 17 y). These circumscribed tumors arose in subcutaneous or deep somatic soft tissue, with a median size of 2.5 cm (range, 1 to 8 cm), being located in the extremities (14/21), trunk (4/21), and upper limb girdle (3/21). Characteristic features included a fibrous pseudocapsule (20/21), peritumoral lymphoplasmacytic infiltrates (20/21), blood-filled cystic spaces (17/21), and prominent myxoid morphology comprising 60% to 100% of the tumor surface area examined. Histiocytoid or spindled tumor cells exhibited vesicular nuclei, inconspicuous nucleoli, palely eosinophilic cytoplasm, and multinodular growth without necrosis. Mucin pools and scattered multinucleate giant cells were observed in a subset of cases. Mild to moderate atypia was observed in 4 cases; 1 tumor showed a pseudochondroid matrix. Immunohistochemically, 14/21 cases expressed desmin, 12/21 expressed EMA, and 4/7 exhibited EWSR1 rearrangement. Follow-up, available for 11 patients (median, 43 mo), revealed that 3 developed local recurrence after 2, 7, and 48 months, respectively. All patients were alive without metastases. AMFH may present with prominent myxoid features making diagnosis difficult and causing possible confusion with other myxoid tumors including low-grade fibromyxoid sarcoma, extraskeletal myxoid chondrosarcoma, and myxoid liposarcoma.

Cutaneous scalp metastases from retroperitoneal leiomyosarcoma: a case report

A 71-year-old woman presented with five scalp nodules that were clinically suspicious for pilar cysts. Histopathologic examination showed a proliferation of mitotically active pleomorphic spindle cells arranged into intersecting fascicles in the dermis and subcutis. Tumor cells displayed deeply eosinophilic cytoplasm and expressed desmin but were negative for S100 protein by immunohistochemistry. Before 10 years, the patient was diagnosed with high-grade retroperitoneal leiomyosarcoma and underwent resection with intraoperative radiation. Metastatic disease involving the lungs, liver and soft tissue developed, requiring treatment with resections, radiation and chemotherapy. Owing to the presentation of multiple scalp nodules with microscopic features of leiomyosarcoma in conjunction with the clinical history of retroperitoneal leiomyosarcoma, a diagnosis of metastatic leiomyosarcoma was made. Scalp metastasis from retroperitoneal leiomyosarcoma is extremely rare and portends a poor prognosis. To our knowledge, only two other cases have been reported in the English literature, and a further search discovered only nine additional cases of scalp metastasis from soft tissue leiomyosarcoma of any non-gynecologic anatomic site. This case highlights the striking microscopic similarity between primary cutaneous and metastatic leiomyosarcoma and illustrates the necessity of adequate clinical information and an appropriate index of suspicion in excluding the possibility of cutaneous metastases of leiomyosarcoma from somatic soft tissue.

Study of Malignant Peripheral Nerve Sheath Tumor in Cerebellopontine Angle

Malignant peripheral nerve sheath tumors (MPNSTs) are very rare soft tissue sarcomas, usually arising from somatic soft tissues or peripheral nerves. Primary MPNST of the cerebellopontine angle is extremely rare, with only a single case reported so far. Here, we report an unusual case of MPNST in cerebellopontine angle in a 25-year-old man presented with dizziness, left facial numbness, and tinnitus. After hospitalization, the tumor was treated with complete surgical excision followed by adjuvant chemotherapy and radiotherapy. Histologically, the tumor showed malignant spindle cells, which were with focal S-100 positivity on immunohistochemistry, and a diagnosis of the MPNST was made. This case is being reported for its rarity and presence in cerebellopontine and illustrated the difficulties in the diagnosis and treatment of MPNST, which to the best of our knowledge, has not been described before in the soft tissue sarcomas.

Primary anterior abdominal wall leiomyoma- a diagnostic enigma.

Primary anterior abdominal wall leiomyoma- a diagnostic enigma.

Metastatic Perivascular Epithelioid Cell Tumor of the Stomach: An Unusual Diagnosis

Purpose: Perivascular epithelioid cell tumor (PEComa) is a very rare mesenchymal neoplasia composed of distinctive perivascular epithelioid cells with variable immunoreactivity for melanocytic and muscle markers. We report a case of metastatic gastric PEComa discovered incidentally in a 74-year-old previously healthy female who underwent an emergent esophagogastroduodenoscopy (EGD) for food impaction. She was noted to have a Schatzki's ring and a 5 mm sessile polyp in the body of the stomach. Polyp biopsies showed a submucosal neoplasm composed of variable polygonal cells with granular eosinophilic cytoplasm. Immunostains showed multifocal positivity for Smooth Muscle Actin and Microphthalmia Transcription Factor. More diffuse positivity noted for desmin and cathepsin-K, while S-100 protein, MART-1, HMB45, c-kit, DOG-1, pan-keratin and CAM5.2 were negative. The tissue morphology and accompanying immunophenotype was most consistent with malignant PEComa. A contrast-CT scan of the abdomen/pelvis showed diffuse liver metastases. PET scan revealed multiple liver lesions, retroperitoneal and abdominal lymphadenopathy, and C5 and S1 bony lesions consistent with metastatic disease. Patient is currently exploring therapeutic options along the lines of metastatic soft tissue sarcoma. Since relatively few cases of malignant PEComa have been reported, firm criteria for malignancy have yet to be established. A recently suggested criteria for malignancy includes: size >5.0 cm, mitotic count >1 per 50 high-power fields and necrosis. Differential diagnosis of PEComa includes gastrointestinal stromal tumors, melanoma, clear cell sarcoma, leiomyosarcoma. PEComas appear to arise most commonly at visceral (especially gastrointestinal and uterine), retroperitoneal, and abdominopelvic sites, with a subset occurring in somatic soft tissue and skin. Malignant PEComa can be very aggressive, leading to multiple metastases and death as expected with a high-grade sarcoma. Surgery seems to be the only approach for aggressive disease, as chemo and radiotherapy has not shown significant results. PEComas show a marked female predominance. At present this neoplasm does not have a known normal cellular counterpart and the natural history is often unpredictable. Our case highlights the fact that despite the small size, the gastric PEComas can behave in an aggressive manner and require careful followup and additional diagnostic evaluation to rule out metastatic disease. Further studies on additional cases with longer clinical follow-up are necessary to accurately predict the biologic behavior of these distinctive tumors.

Novel YAP1‐TFE3 fusion defines a distinct subset of epithelioid hemangioendothelioma

Conventional epithelioid hemangioendotheliomas (EHE) have a distinctive morphologic appearance and are characterized by a recurrent t(1;3) translocation, resulting in a WWTR1-CAMTA1 fusion gene. We have recently encountered a fusion-negative subset characterized by a somewhat different morphology, including focally well-formed vasoformative features, which was further investigated for recurrent genetic abnormalities. Based on a case showing strong transcription factor E3 (TFE3) immunoreactivity, fluorescence in situ hybridization (FISH) analysis for TFE3 gene rearrangement was applied to the index case as well as to nine additional cases, selected through negative WWTR1-CAMTA1 screening. A control group, including 18 epithelioid hemangiomas, nine pseudomyogenic HE, and three epithelioid angiosarcomas, was also tested. TFE3 gene rearrangement was identified in 10 patients, with equal gender distribution and a mean age of 30 years old. The lesions were located in somatic soft tissue in six cases, lung in three and one in bone. One case with available frozen tissue was tested by RNA sequencing and FusionSeq data analysis to detect novel fusions. A YAP1-TFE3 fusion was thus detected, which was further validated by FISH and reverse transcription polymerase chain reaction (RT-PCR). YAP1 gene rearrangements were then confirmed in seven of the remaining nine TFE3-rearranged EHEs by FISH. No TFE3 structural abnormalities were detected in any of the controls. The TFE3-rearranged EHEs showed similar morphologic features with at least focally, well-formed vascular channels, in addition to a variably solid architecture. All tumors expressed endothelial markers, as well as strong nuclear TFE3. In summary, we are reporting a novel subset of EHE occurring in young adults, showing a distinct phenotype and YAP1-TFE3 fusions.

Characteristics, changes and influence of body composition during a 4486 km transcontinental ultramarathon: results from the TransEurope FootRace mobile whole body MRI-project.

BackgroundAlmost nothing is known about the medical aspects of runners doing a transcontinental ultramarathon over several weeks. The results of differentiated measurements of changes in body composition during the Transeurope Footrace 2009 using a mobile whole body magnetic resonance (MR) imager are presented and the proposed influence of visceral and somatic adipose and lean tissue distribution on performance tested.Methods22 participants were randomly selected for the repeated MR measurements (intervals: 800 km) with a 1.5 Tesla MR scanner mounted on a mobile unit during the 64-stage 4,486 km ultramarathon. A standardized and validated MRI protocol was used: T1 weighted turbo spin echo sequence, echo time 12 ms, repetition time 490 ms, slice thickness 10 mm, slice distance 10 mm (breath holding examinations). For topographic tissue segmentation and mapping a modified fuzzy c-means algorithm was used. A semi-automatic post-processing of whole body MRI data sets allows reliable analysis of the following body tissue compartments: Total body volume (TV), total somatic (TSV) and total visceral volume (TVV), total adipose (TAT) and total lean tissue (TLT), somatic (SLT) and visceral lean tissue (VLT), somatic (SAT) and visceral adipose tissue (VAT) and somatic adipose soft tissue (SAST). Specific volume changes were tested on significance. Tests on difference and relationship regarding prerace and race performance and non-finishing were done using statistical software SPSS.ResultsTotal, somatic and visceral volumes showed a significant decrease throughout the race. Adipose tissue showed a significant decrease compared to the start at all measurement times for TAT, SAST and VAT. Lean adipose tissues decreased until the end of the race, but not significantly. The mean relative volume changes of the different tissue compartments at the last measurement compared to the start were: TV −9.5% (SE 1.5%), TSV −9.4% (SE 1.5%), TVV −10.0% (SE 1.4%), TAT −41.3% (SE 2.3%), SAST −48.7% (SE 2.8%), VAT −64.5% (SE 4.6%), intraabdominal adipose tissue (IAAT) −67.3% (SE 4.3%), mediastinal adopose tissue (MAT) −41.5% (SE 7.1%), TLT −1.2% (SE 1.0%), SLT −1.4% (SE 1.1%). Before the start and during the early phase of the Transeurope Footrace 2009, the non-finisher group had a significantly higher percentage volume of TVV, TAT, SAST and VAT compared to the finisher group. VAT correlates significantly with prerace training volume and intensity one year before the race and with 50 km- and 24 hour-race records. Neither prerace body composition nor specific tissue compartment volume changes showed a significant relationship to performance in the last two thirds of the Transeurope Footrace 2009.ConclusionsWith this mobile MRI field study the complex changes in body composition during a multistage ultramarathon could be demonstrated in detail in a new and differentiated way. Participants lost more than half of their adipose tissue. Even lean tissue volume (mainly skeletal muscle tissue) decreased due to the unpreventable chronic negative energy balance during the race. VAT has the fastest and highest decrease compared to SAST and lean tissue compartments during the race. It seems to be the most sensitive morphometric parameter regarding the risk of non-finishing a transcontinental footrace and shows a direct relationship to prerace-performance. However, body volume or body mass and, therefore, fat volume has no correlation with total race performances of ultra-athletes finishing a 4,500 km multistage race.

English

Malignant peripheral nerve sheath tumors (MPNSTs) are rare neoplasms, usually arising from somatic soft tissues or peripheral nerves. The incidence of MPNSTs is approximately 0.001% in the general population. MPNSTs are mainly located in the buttocks, thighs, brachial plexus, and paraspinal region. Scalp is an unusual and surgically challenging site of occurrence. We report a case of giant recurrent MPNST in the left fronto-parietal region, with intracranial extension, in a 58 year old female which was excised completely along with the involved overlying skin, and reconstruction was done for the bony and skin defect. She was given adjuvant radiotherapy. She is doing well after treatment and is in regular follow up. MPNST should be considered by neurosurgeons in the differential of an enlarging scalp soft-tissue lesion with bony and intracranial involvement. Scalp MPNSTs are mostly aggressive lesions, and multimodality approaches are necessary to optimize outcomes   Key words: Malignant peripheral nerve sheath tumors (MPNST), scalp, intracranial, surgery.

Rhabdomyosarcoma in middle to old-aged patients: analysis of clinicopathological features and prognosis in 76 cases

Albeit rare, rhabdomyosarcoma (RMS) may occur in middle to old-aged adults. The purpose of this study was to investigate the clinicopathological characteristics and prognosis of RMS in patients ≥ 40 years of age. The clinical, pathological and immunohistochemical profiles of the 76 cases were reviewed, and the outcomes were analyzed. There were 46 males and 30 females with a median age of 55 years. Sixty cases occurred in somatic soft tissues, including extremities (n = 25), head and neck (n = 19) and trunk (n = 16). Sixteen developed in the internal organs with predilection for the urogenital tract. Histologically, 38 cases (50.0%) were of embryonal RMS (ERMS) subtype, 29 cases (38.2%) of pleomorphic RMS (PRMS), 7 cases (9.2%) of poorly-differentiated RMS (PdRMS), and 2 cases (2.6%) of alveolar RMS (ARMS). Immunohistiochemically, the tumor cells showed diffuse staining of desmin (68/68, 100%), with a variable expression of myogenin (30/43, 69.8%), MyoD1 (33/51, 64.7%) and MSA (29/40, 72.5%). The therapy data available in 54 cases showed that three inoperable cases were treated with chemotherapy and radiotherapy, 51 cases underwent local excision, including 10 cases with chemotherapy, 9 cases with radiotherapy, 8 cases with chemotherapy and radiotherapy after surgery, and the remaining 24 cases were not administrered with postoperative adjuvant therapy. The follow-up data available in 54 cases showed that 22 patients were alive with unresectable or recurrent/disease and 32 patients were alive with no evidence of disease. The median disease-free and overall survivals were 6.0 months and 7.0 months, respectively. Twenty-seven patients exhibited progression of disease with recurrence in 8 cases and metastasis in 19 cases. The median interval to progression was 6.0 months. The most common subtypes of RMS in middle to old aged patients are ERMS and PRMS. In general, Rhabdomyosarcomas of middle to old aged patients shows a highly aggressive behavior. Patients may benefit from multi-disciplinary therapy.

Giant Primary Mesenchymal Chondrosarcoma of the Lung: Case Report and Review of Literature

Mesenchymal chondrosarcoma, a rare malignant tumor, was predominantly occurring in the bone and may involve somatic soft tissue but it is extremely rare in the lung.We report the case of a 20-year-old female who presented with a 2-month history of irritant nonproductive cough and chest pain. The histopathologic examination revealed the tumor composed of atypical undifferentiated small cells and islands of matured chondroid matrix typically presented as bimorphic appearances. Immunohistochemical examination revealed that the tumor cells were positive for vimentin and CD99 for all components, and to S-100 limited to the areas of cartilage. In addition, previously reported cases of primary lung mesenchymal chondrosarcoma were reviewed, and the relevant clinical knowledge regarding its clinical manifestations, diagnosis, and treatment were discussed.

Primary pseudomyogenic haemangioendothelioma of bone

Pseudomyogenic haemangioendothelioma is a recently described vascular neoplasm that occurs most commonly in the soft tissue of distal extremities of young adults. Occurrence outside the somatic soft tissue has not been described thus far. We present here a unique case of pseudomyogenic haemangioendothelioma that arose in the long tubular bones of the lower extremity. The initial open biopsy was interpreted as a fibrous histiocytoma. However, the curettage specimen showed prominent epithelioid cytomorphology with a striking rhabdomyoblast-like appearance. By immunohistochemistry, the linear membranous staining of CD31 was highly suggestive of endothelial differentiation of the tumour cells. To the best of our knowledge, this case represents the first example of primary pseudomyogenic haemangioendothelioma of bone. Clinical and pathological correlation with application of immunohistochemistry is mandatory in establishing the correct diagnosis and excluding tumours with overlapping features.

Imaging findings of solitary fibrous tumors

Objective To investigate the imaging characteristics of solitary fibrous tumor(SFT).Methods This study included 11 cases with SFT proved by pathological results.The imaging manifestations were retrospectively reviewed and analyzed in comparison with surgical and pathological results. Among them,8 patients underwent CT plain scan (1/8) or enhanced scan (7/8),4 patients underwent MR plain scan (2/4) or enhanced scan (2/4),and only 1 patient underwent both CT and MR scan.Results ( 1 )SFT were located in the thoracic cavity(2/11 ),abdominal and pelvis(5/11 ),and somatic soft tissues (4/11 ).The average tumor size was 11.8 cm ( 2.5 to 23.0 cm).Among them,4 tumors were round or ellipse,and 7 tumors were irregular.Eight tumors had well-defined contours,and the others showed illdefined contours.(2)On CT scan,all SFTs showed inhomogeneous density with low density areas.Calcification was seen in I case,and hemorrhagic foci were detected in 2 cases.(3)On MR scan,all SFTs showed intermediate signal intensity on T1 WI.Three tumors were slightly hyperintense and the other was hypointense on T2WI.All of them were rich of vascular signal voids.Three tumors,including 2 tumors with scattered long T1 and T2 signal and 1 tumor in the liver with hyperintense haemorrhage,displayed inhomogeneous mixed signal.One tumor in the orbit showed homogeneous signal.(4)On enhanced scan,9 tumors showed irregular enhancement with multiple circuitous vessels in the arterial phase,and continuous or progressive enhancement with patch non-enhanced areas in the venous phase.( 5 ) Histologically,SFTs were composed of juxtaposed hyper- and hypo-cellular spindle cells,dense collagenous stromas and numerous thin-walled blood vessels with a staghorn configuration.Conclusion The possibility of SFT should be considered when a single soft tissue mass with sharp border,inhomogeneous density is detected,especially with inhomogeneous enhancement maintaining in the venous phase. Key words: Fibroma; Tomography,X-ray computed; Magnetic resonance imaging

Leiomyosarcoma of the Somatic Soft Tissues

Leiomyosarcomas of the somatic soft tissues are tumors of smooth muscle origin that occur in the extremities. These lesions are commonly high-grade tumors that carry a poor prognosis. Recommended treatment often includes wide excision and chemotherapy or radiation therapy. Sixty-five patients were followed for a mean of 4.1 years. The mean maximum tumor diameter was 7 cm, and approximately 70% of all patients had tumors deep to fascia. Including all stages of disease, the overall 1-, 2-, and 5-year survival rates were 91%, 87%, and 68%, respectively. Mitotic rate and tumor depth were significant predictors of development of recurrent disease and metastatic disease. Tumor size was another predictor of recurrent disease. The mitotic rate and AJCC stage were also important predictors of overall survival. Patients with deep lesions, histologic grade 3 disease/higher mitotic rates, and advanced stage of disease had a poorer prognosis and thus were more likely to undergo adjuvant chemotherapy. Future clinical studies may help determine if knowledge of these predictors can help guide treatment and improve clinical outcomes.