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Pseudomyogenic hemangioendothelioma (PHE) is a rare neoplasm with vascular origin mostly seen on the extremities. Up to date, the terminology which represents PHE has been a subject to debate. According to the World Health Organization, PHE is classified among the soft tissue tumors. PHE is mostly seen in soft tissue planes and the distal lower extremities are the most affected location of the body. Localization of this tumor on the head and neck region is uncommon. PHE, being a sarcoma does not favor lymphogenic metastasis. As far as we are concerned, this is the first described case of PHE of the external ear and retro auricular sulcus. In this paper, the management of this case and the current treatment options of PHE are discussed. [ABSTRACT FROM AUTHOR]

Ectomesenchymal chondromyxoid tumor is a rare and benign neoplasm with a predilection for the anterior dorsal tongue. Despite morphologic heterogeneity, most cases are characterized by a proliferation of bland spindle cells with a distinctive reticular growth pattern and myxoid stroma. The immunophenotype of these neoplasms is likewise variable; most cases express glial fibrillary acid protein and S100 protein, with inconsistent reports of keratin and myoid marker expression. The molecular pathogenesis is poorly understood; however, a subset of cases has been reported to harbor EWSR1 gene rearrangement. Following identification of an RREB1-MKL2 fusion gene by RNA Sequencing in an index patient, a retrospective review of additional cases of ectomesenchymal chondromyxoid tumors was performed to better characterize the clinical, immunohistochemical, and molecular attributes of this neoplasm. A total of 21 cases were included in this series. A marked predisposition for the dorsal tongue was confirmed. Most cases conformed to prior morphologic descriptions; however, hypercellularity, hyalinized stroma, and necrosis were rare attributes not previously emphasized. The neoplastic cells frequently coexpressed glial fibrillary acid protein, S100 protein, keratin, smooth muscle actin, and/or desmin; a single case was found to contain significant myogenin expression. An RREB1-MKL2 fusion product was identified in 19 tumors (90%), a single tumor (5%) had an EWSR1-CREM fusion product, and the remaining case lacked any known fusion gene by RNA Sequencing. The latter 2 cases subtly differed morphologically from many in the cohort. This series illustrates that recurrent RREB1-MKL2 fusions occur in most, perhaps all, cases of ectomesenchymal chondromyxoid tumor.

Objective: Rare disease Background: Sarcomas account for less than 1% of all cancers. Undifferentiated Pleomorphic Sarcoma, formerly called Malignant Fibrous Histiocytoma, is a rare subtype identified by a lack specific immunohistochemical markers for a specific lineage of differentiation. These soft tissue tumors are aggressive and rapidly enlarge. Risk for metastasis increases almost linearly as the tumor increases in size, emphasizing the importance of early detection, treatment, and post-resection monitoring. Case Report: This article reports a case of a large undifferentiated pleomorphic sarcoma of the posterior thigh in a 62-yearold female. Given the patient's history of thrombotic thrombocytopenic purpure, her initial mass was thought to be a hematoma following a hernia repair surgery. After diagnosis of undifferentiated pleomorphic sarcoma, she underwent radical excision revealing a 24×9.5×7cm lesion - one of the largest reported in the literature. Conclusions: Sarcomas are very rare soft tissue neoplasms, but they should not be excluded in a physician's differentials when a patient presents with an enlarging soft tissue mass. Because sarcomas enlarge rapidly, delay in evaluation and management should be avoided and these patients should be quickly referred to a center specializing in sarcoma treatment. Magnetic Resonance Imaging (MRI) is the recommended initial imaging for all soft tissue masses of the extremities, trunk, and head and neck while Computed Tomography (CT) is the recommended imaging choice for retroperitoneal and visceral masses. After successful surgical excision with clean margins, patients should undergo serial monitoring by CT or MRI for surveillance of recurrence or late pulmonary metastases. [ABSTRACT FROM AUTHOR]

Purpose: To describe the clinicopathological and morphological features of gastrointestinal stromal tumours (GISTs) on multi-detector computed tomography (MDCT). Material and methods: MDCT (plain and post contrast) images of 25 confirmed cases of GISTs were retrospectively evaluated from our hospital database. The images were analysed for the location, size, growth pattern, attenuation pattern, relation to adjacent structures, presence or absence of ulceration, calcification, metastases, lymphadenopathy, and for any complications such as haemorrhage, intestinal obstruction, etc. Institutional Ethics Committee clearance was obtained prior to the commencement of the study. Statistics used included percentage frequency. Results: Our study group comprised 14 males and 11 females. The mean age of our study population was 60 years (age range: 40 to 82 years). The mean tumour size was 11.7 cm. The stomach and small bowel accounted for 76% of the primary tumour site. The commonest imaging appearance of GIST observed in our study was that of an exophytic mass (76%) with a heterogenous pattern of enhancement (96%) with intratumoural necrosis (76%). MDCT demonstrated complications in three patients (12%). Six patients presented with metastatic foci (five to the liver and one to the lung), while lymphadenopathy was observed in five patients (20%). Associated complications included intestinal obstruction (8%) and retrogastric haematoma (4%). Incidental findings included uterine fibroid (n = 1), ovarian dermoid (n = 1), and chronic pancreatitis (n = 1). Conclusions: GISTs are predominantly large tumours with a well circumscribed and exophytic pattern on MDCT, with or without cystic/necrotic areas, and they mostly show a heterogenous pattern of enhancement on post-contrast administration. [ABSTRACT FROM AUTHOR]

Introduction: The aims of this study were to report the presenting characteristics and identify how best to distinguish bone and soft-tissue infections that mimic sarcomas., Materials and Methods: A total of 238 (211 osteomyelitis and 27 soft-tissue infections) patients referred to a tertiary sarcoma multidisciplinary team with suspected sarcoma who were ultimately diagnosed with a bone or soft tissue infection were included. Data from a prospectively collated database was analysed retrospectively., Results: Of all possible bone and soft-tissue sarcoma referrals, a diagnosis of infection was made in 2.1% and 0.7%, respectively. Median age was 18 years in the osteomyelitis group and 46 years in the soft-tissue infection group. In the osteomyelitis group, the most common presenting features were pain (85.8%) and swelling (32.7%). In the soft-tissue infection group, the most common clinical features were swelling (96.3%) and pain (70.4%). Those in the soft-tissue group were more likely to have raised inflammatory markers. Radiological investigations were unable to discern between tumour or infection in 59.7% of osteomyelitis and 81.5% of soft-tissue infection cases. No organism was identified in 64.9% of those who had a percutaneous biopsy culture., Conclusions: This study has highlighted that infection is frequently clinically indistinguishable from sarcoma and remains a principle non-neoplastic differential diagnosis. When patients are investigated for suspected sarcoma, infections can be missed due to falsely negative radiological investigations and percutaneous biopsy. As no single clinical, biochemical or radiological feature or investigation can be relied upon for diagnosis, clinicians should have a low threshold for tissue biopsy and discussion in a sarcoma multidisciplinary team meeting.