Vocal cord inflammatory myofibroblastic tumor with mucoid deposits harboring TIMP3–ALK fusion: A potential diagnostic pitfall

A 35‐year‐old Japanese man who had experienced hoarseness for 10 years presented with a vocal cord lesion. A gross examination revealed a left vocal cord polyp occupying two‐thirds of the vocal space. The endoscopically resected lesion contained scattered atypical fibroblastic, stellate, or ganglion‐like cells with mucoid stroma. Vacuolated cells were also seen. Lymphoplasmacytic infiltrate was largely undetectable. A vocal cord polyp was first suspected, but well‐differentiated liposarcoma and inflammatory myofibroblastic tumor (IMT) were included in the differential diagnoses. The tumor cells were positive for anaplastic lymphoma kinase (ALK), calponin, and vimentin, and negative for other smooth muscle markers by immunohistochemistry. Structures resembling myofibroblasts were not observed by electron microscopy, which confirmed abundant rough endoplasmic reticulum in the tumor cells and accumulated lipid droplets in some tumor cells. ALK gene rearrangement was detected by fluorescence in situ hybridization, and TIMP3–ALK fusion was confirmed by 5′ rapid ampli?cation of cDNA ends. We diagnosed the lesion as an IMT, and an ALK‐rearranged stellate cell tumor may be postulated. This is the first report of a fusion partner gene of ALK in a case of laryngeal IMT.     

梭形细胞脂肪肉瘤的临床病理分析

目的分析梭形细胞脂肪肉瘤的临床病理特点、诊断及鉴别诊断。方法对12例梭形行细胞脂肪肉瘤进行HE染色、免疫组织化学检测并分析病理学特征。结果肿瘤镜下排列成旋涡状或束状,一些成熟的脂肪细胞中散在大小不一的异型细胞;免疫组织化学结果显示梭形细胞Vimentin阳性,S-100、MSA部分阳性,MBP、HMB45、Desmin阴性,脂肪细胞及核深染的异型细胞呈S-100阳性。结论梭形细胞脂肪肉瘤一种少见软组织恶生肿瘤,需与多种软组织肿瘤鉴别。     

去分化型脂肪肉瘤的临床病理--附2例报告及文献复习

目的探讨去分化型脂肪肉瘤的临床病理特点、诊断和鉴别诊断要点。方法报告2例发生于四肢软组织去分化型脂肪肉瘤患者的临床和病理资料,并复习相关文献。结果例1男,59岁,左上臂肿胀2个月余,无明显疼痛;例2女,62岁,右股部肿物1个月余。2例均予单纯肿瘤切除术,术后随访3个月和6个月,肿瘤无复发。病理检查见分化好的脂肪肉瘤和高度恶性非脂肪源性的肉瘤并存,分化好的区域含有脂肪瘤样、硬化性或者粘液样脂肪肉瘤成分,非脂肪源性肉瘤成分类似于恶性纤维组织细胞瘤,两者有明显的移行。免疫组织化学染色显示:瘤细胞S-100蛋白、CD34阳性,CD68、SMA、CD31、Actin阴性。结论去分化型脂肪肉瘤具有较高的复发率,易误诊为恶性纤维组织细胞瘤,其诊断主要依据组织病理学,外科手术切除是主要的治疗手段。     

Spermatic cord liposarcomas incidentally found during hernia surgery: is histology of any lipoma mandatory? A review of the literature

Abstract

Purpose: Liposarcomas found incidentally during open or laparoscopic inguinal hernia surgery are extremely rare. It is unclear, whether any adipose tissue being removed during inguinal hernia surgery must be sent for histology due to the potential risk of liposarcoma of the spermatic cord. This study aims to evaluate the frequency of liposarcomas incidentally found in the inguinal canal during hernia surgery and tries to derive evidence-based recommendations regarding the optimal management of any fatty tissue found in the inguinal canal.

Methods: A literature review of the PubMed/Medline electronic databases between January 1980 and January 2019 was performed using the search terms ‘inguinal hernia’ and ‘liposarcoma’. There was only one study available on this topic. Therefore, an additional literature review was performed analyzing all reports on patients with incidentally detected liposarcomas of the spermatic cord in the inguinal canal during hernia surgery.

Results: There was only one retrospective study evaluating the frequency of inguinal liposarcoma found at hernia operations with a frequency of less than 0.1%. There were 18 cases of spermatic cord liposarcomas that were truly found incidentally during operation for an unsuspected symptomatic or incarcerated inguinal hernia. These included 16 case reports with a total of 18 patients and 19 liposarcomas. All patients were male with a median age of 62.5?years (range: 24–86?years) years. Median size of liposarcoma was 10.5?cm (range: 3–30?cm). In seven patients, the inguinal liposarcoma was an extension of a retroperitoneal sarcoma. Treatment consisted of radical orchidectomy during the primary operation in 12 patients. Three out of the seven patients with retroperitoneal extension of the tumor underwent a secondary operation with complete resection of the tumor.

Conclusions: Currently, there is no evidence-based recommendation available regarding the management of lipomas detected during open or laparoscopic inguinal hernia surgery. Due to the extremely low risk of the presence of a liposarcoma, routine histologic examination cannot be recommended unless the diameter exceeds 10?cm.     

Primary liposarcoma of the mediastinum —A case report and review of the literature—

Liposarcoma of the mediastinum is a rare disease. A 59 year old woman was seen with the complaint of dizziness. A chest radiogram taken during the course of investigation for hypertension revealed a large mass shadow 24×18.5×12 cm located in the anterior mediastinum. The tumor of the mediastinum was diagnosed as a benign lipoma when it was first resected, but subsequently proved to be a liposarcoma when it re-appeared 2 years and 10 month later. Based on the WHO classification, the mixed type of liposarcoma was diagnosed. The salient and pathological features of mediastinal liposarcoma are reviewed and compared with those nine cases reported in Japan and fifty in North America and European countries. Treatment by simple enucleation or shelling out of the tumor should be discouraged, since this seems to be the main cause of local recurrence. Surgical wideen bloc excision is the treatment of choise.     

Myxoid liposarcoma mimicking fluid density

A case of liposarcoma of the liver capsule initially presenting as massive pleural effusion and ascites is reported. The chest radiograph, computed tomographic scan, and sonogram are emphasized.     

Myxoid liposarcoma of the broad ligament

A patient diagnosed as having a myxoid liposarcoma of the broad ligament is presented. She was treated with surgical resection and radiation therapy and is free of disease 2 years after completing surgery.     

Early detection of multiple bone and extra-skeletal metastases by body magnetic resonance imaging (BMRI) after treatment of Myxoid/Round-Cell Liposarcoma (MRCLS)

IntroductionMyxoid Round cell containing myxoid liposarcomas (MRCLS) have a high propensity to metastasize to soft tissue and bone. Whole Body Magnetic Resonance Imaging (BMRI) has been reported as a critical modality to early detect disease spreading in asymptomatic patients. The purpose of this study is to describe metastatic patterns and outcomes in patients through annual BMRI surveillance after diagnosis of MRCLS of the extremities and trunk.Materiel and patientsThis retrospective study included patients with histology confirmed MRCLS. Initial BMRI were done within 6 months following the first line treatment then once a year.ResultsForty-five out of 51 consecutive MRCLS patients were included. At the last follow-up 10 patients (22.2%) had an extra-pulmonary soft-tissue or/and bone metastasis detected in a median delay of 22.7±16 months [0−49] from the diagnosis of the MRCLS. Nine patients were asymptomatic. Finally, 5-years metastatic free survival was 72±8%. All metastatic patients had multiple lesion within the year following the first lesion diagnosis.ConclusionSystematic BMRI in MRCLS patients following treatment frequently identify extra-pulmonary metastasis in asymptomatic patients within the first 5 years of follow-up. Despite a long survival can be expected after diagnosis, extra-skeletal metastasis was a signal of disseminated disease.     

Imaging diagnosis of abdominal and pelvic sarcomas

Sarcomas of the abdomen and pelvis are rare malignant mesenchymal neoplasms that often remain undiagnosed until they attain large size. Complete tumour resection is associated with improved patient survival. Detection and delineation of the tumour, its extent and its relationship with surrounding tissues are important for surgical planning. Radiography, barium studies, and intravenous urography are of limited value in the imaging diagnosis of abdominal and pelvic sarcomas. Ultrasound is sensitive for detecting neoplasms larger than 6 cm. Computed tomography is useful for detection, defining extent, and predicting resectability of the primary tumour, evaluation of response to treatment, and detecting recurrence and metastasis. Sarcomas are predominantly large, poorly demarcated, heterogeneous masses of muscle density with haemorrhage or necrosis.