Case Of Undifferentiated Pleomorphic Sarcoma Research Articles

A case of undifferentiated pleomorphic sarcoma arising during follow-up of the vocal cord dysplasia

A case of undifferentiated pleomorphic sarcoma arising during follow-up of the vocal cord dysplasia

A Case of Undifferentiated Pleomorphic Sarcoma of the Mesentery of the Ascending Colon

A Case of Undifferentiated Pleomorphic Sarcoma of the Mesentery of the Ascending Colon

Preoperative evaluation of the efficacy of radio-hyperthermo-chemotherapy for soft tissue sarcoma in a case series.

PurposeRadio-hyperthermo-chemo (RHC) therapy, which combines radiotherapy, hyperthermia, and chemotherapy, for malignant soft tissue tumors has been introduced with the aim of decreasing the possibility of local recurrence after surgery. To avoid unnecessary neoadjuvant therapy and to plan the appropriate surgical treatment, surveillance of RHC therapeutic efficacy during treatment is necessary. In this study, we determined the optimal response criteria to evaluate the efficacy of RHC by comparing preoperative images before and after RHC with pathological evaluation of necrosis in the resected tumor.Patients and methodsFrom 2004 to 2014, 20 patients were enrolled into this study. Needle biopsy revealed 6 cases of myxoid liposarcoma, 6 cases of undifferentiated pleomorphic sarcoma, 4 cases of myxofibrosarcoma, and 4 cases of synovial sarcoma. Based on the Response Evaluation Criteria in Solid Tumors (RECIST) 1.1 or modified RECIST, we calculated the responses to RHC therapy by comparing pre- and post-RHC therapy images. In addition, resected specimens underwent pathological analysis to evaluate response based on tumor necrosis. The correlation between assessment based on preoperative images and resected tumors were evaluated by the Spearman’s rank-order correlation coefficient.ResultFrom the surgical specimens, pathological assessment of necrosis in resected tumor were assessed as less than 50% (2 cases), 50–90% (9 cases), 90–99% (6 cases), and total necrosis (3 cases). Use of the RECIST 1.1 underestimated good responders as stable disease (SD) or progressive disease (PD) in 5 out of 15 cases; on the other hand, use of the modified RECIST did not underestimate the pathological assessment of necrosis. The correlations between responses based on preoperative images and those based on histological assessments were 0.23 (RECIST 1.1) and 0.76 (modified RECIST).ConclusionBecause pathological responses can be underestimated using the RECIST 1.1, the modified RECIST, which take into consideration tumor viability, as assessed by contrast MRI, should also be considered when evaluating the efficacy of RHC.

A case of undifferentiated pleomorphic sarcoma in the sacrum treated by carbon ion radiotherapy

A case of undifferentiated pleomorphic sarcoma in the sacrum treated by carbon ion radiotherapy

A Case of Undifferentiated Pleomorphic Sarcoma of the Neck

A Case of Undifferentiated Pleomorphic Sarcoma of the Neck

脱分化型脂肪肉腫と鑑別を要した未分化多形肉腫の 1 例

69 歳，男性。当科初診の半年前に背部の腫瘤に気づき，その後急速に増大したため近医を受診した。 皮膚生検の結果，悪性線維性組織球腫 (malignant fibrous histiocytoma : MFH) が疑われ，切除目的に当科紹介となった。広範切除時の病理組織像で，多形性を有する肉腫の像を認め，免疫組織化学染色から MDM2 (murine double minute-2) 陰性および CDK4 (cyclin dependent kinase-4) 陽性の結果が得られたため，脱分化型脂肪肉腫 (dedifferentiated liposarcoma : DDLS) の可能性も否定できないと考えた。しかし，最近の知見をもとに症例を再度検討したところ，FISH (fluorescence in situ hybridization) 法にて，MDM2 遺伝子増幅を認めず，未分化多形肉腫 (undifferentiated pleomorphic sarcoma : UPS) と再診断した。免疫組織化学染色において，MDM2，CDK4 は上記 2 疾患の鑑別に有用な検査であるが，脂肪肉腫では MDM2 より CDK4 の感度が高いことが知られている一方，CDK4 の特異性には議論がある。

Undifferentiated pleomorphic sarcoma of the left parotid gland: a case report

Undifferentiated pleomorphic sarcoma, formerly known as malignant fibrous histiocytoma, was previously the most common type of soft-tissue sarcoma in adults (40 %). Undifferentiated pleomorphic sarcomas or malignant fibrous histiocytomas comprise a group of high-grade pleomorphic sarcomas that cannot be otherwise classified. After a re-classification, the term malignant fibrous histiocytoma became a diagnosis of exclusion. It was first recognized as a distinct clinicopathologic entity in the early 1960s as pleomorphic sarcoma. In the head and neck, malignant fibrous histiocytoma is extremely rare and accounts for only 3 % of all undifferentiated pleomorphic sarcomas. We report an unusual case of undifferentiated pleomorphic sarcoma involving the left parotid gland in a 19-year-old male patient.

Undifferentiated Pleomorphic Sarcoma of the Thoracic Aorta: A Case Report

Primary sarcomas of the great vessels are extremely uncommon tumors, with aortic sarcoma being the rarest type. They tend to occur in the thoracic aorta, followed by the abdominal aorta and the thoracoabdominal aorta. They have a propensity for metastases and they cause acute arterial embolism (1). Undifferentiated pleomorphic sarcoma, also called malignant fibrous histiocytoma, has been defined as a group of pleomorphic, high-grade sarcomas that fail to show any line of differentiation using currently available ancillary techniques (2). To the best of our knowledge, no fewer than 24 cases of undifferentiated pleomorphic sarcoma originating from the aorta have been reported in the worldwide literature, but imaging findings have been rarely reported (3-7). Herein, we describe the case of a 67-year-old male who had a primary undifferentiated pleomorphic sarcoma of the descending thoracic aorta with an emphasis on computed tomography (CT) and magnetic resonance imaging (MRI) findings. CASE REPORT

上行結腸間膜原発未分化多形肉腫の1例

症例は78歳，男性．持続する発熱と全身倦怠感のため入院．右下腹部に手拳大の腫瘤を触知し，腹部CTで上行結腸内側に境界明瞭，内部に低吸収領域を伴う7cm大の腫瘤を認めた．下部消化管内視鏡検査で上行結腸に粘膜下腫瘍様の病変を認めたが，確定診断に至らなかった．発熱とCRP高値，肝機能異常も持続し保存的治療で改善がみられなかったため，診断目的も兼ねて開腹手術を行った．約7cm大の腫瘤を上行結腸間膜内に認め，回盲部切除術を施行した．病理組織学的検査の結果，腸間膜原発と考えられる未分化多形肉腫と診断された．術後補助化学療法は行わず，現在術後1年7カ月経過したが無再発生存中である．本疾患は症例報告も少なく，その特徴は未だ明らかにされていないが，完全切除によって長期予後が得られる可能性があるため，根治的な手術を心がけることと，更なる症例の蓄積が重要と思われた．

Undifferentiated Pleomorphic Sarcoma of Larynx: A Rare Entity with Review of Literature

Sarcomas of the larynx are rare neoplasms that constitute less than 1% of laryngeal malignancies. We describe a rare case of undifferentiated pleomorphic sarcoma of larynx of a 62-year- old male who presented with hoarseness of voice. Laryngoscope showed growth over glottic region of larynx and biopsy revealed poorly differentiated carcinoma. Patient was treated with radical radiotherapy (RT). After 3 months of completion of RT, laryngoscopy and CECT revealed residual lesion. Cordectomy was done. Post- cordectomy histopathology report (HPR) and review of pre RT biopsy specimen showed tumour composed of highly pleomorphic spindle cells with moderate eosinophilic cytoplasm, vesicular nuclei with prominent eosinophilic nucleoli, frequent mitoses and occasional multinucleate cells. IHC showed tumour cells were positive for vimentin and focal positive for smooth muscle actin but were negative for Pan-CK, CK- 5/6, S-100, CD-34 and desmin. The overall features were consistent with undifferentiated pleomorphic sarcoma. Postcordectomy PET-CT showed metastatic disease. Patient was further managed with 6 cycles of Ifosfamide and Epirubicin based palliative chemotherapy (CT) and after 6 months of CT completion, patient is symptomatically better. The characteristic clinical, histopathological features and management of this rare case are described with a literature review.

縦隔内転移を生じた未分化多形型肉腫の1例

縦隔内転移を生じた未分化多形型肉腫の1例

Paratesticular sarcomas: A report of seven cases

Primary tumors of the paratesticular region are rare, with paratesticular sarcomas constituting a major proportion of these tumors, particularly in the elderly. The paratesticular region consists of mesothelial, various epithelial and mesenchymal cells and may therefore give rise to a number of tumors with various behaviors. Defining the association between the paratesticular mass and the testicle, and differentiation between benign and malignant masses using radiology is challenging, therefore the mass is usually considered to be malignant and radical orchiectomy with high ligation is performed. The present study reports the cases of seven patients with tumors of the paratesticular region and presents the clinical and significant histological features of the tumors. In total, two patients suffered from dedifferentiated liposarcoma (DDLS), two exhibited leiomyosarcoma, two exhibited low-grade fibromyxoid sarcoma and one case of undifferentiated pleomorphic sarcoma was identified. Radical orchiectomy with high ligation was performed in five cases; simple orchiectomy was performed in one case and excisional biopsy was performed in the remaining case. A leiomyosarcomatous and epithelial membrane antigen (EMA) positive whorl pattern was observed during microscopy in the two DDLS cases. Additionally, one of the low-grade fibromyxoid sarcoma patients exhibited pleomorphism and mitosis in focal areas. To the best of our knowledge, the present study is the second time low-grade fibromyxoid sarcoma cases with paratesticular localization have been reported in the literature. Of the seven cases, four patients succumbed to the disease, one patient is living with the disorder and the two cases of DDLS are living without the disease. Paratesticular sarcomas are often aggressive and a multidisciplinary approach is required for the diagnosis and treatment of these tumors.

腹腔鏡を併用して切除した腹直筋直下未分化多形肉腫の1例

腹腔鏡を併用して切除した腹直筋直下未分化多形肉腫の1例

An unusual case of metastasis of a pulmonary undifferentiated pleomorphic sarcoma to the right ventricle: a case report

IntroductionUndifferentiated pleomorphic sarcoma is defined as a pleomorphic high-grade sarcoma whose line of differentiation cannot be determined. These tumors constitute less than 5% of all sarcomas in adults. Cardiac neoplasms are rare, and most are metastatic in origin. More than one-third of cardiac metastases originate from lung cancer. Symptoms of cardiac neoplasms usually appear late in the course of the disease and are often ignored because of the more severe effects of the primary malignancy or its therapy. We present the case of a patient with undifferentiated pleomorphic sarcoma of the lung presenting with symptomatic right-heart failure secondary to cardiac metastasis. The purpose of this report is to present this unusual case.Case presentationOur patient was a 59-year-old Chinese woman with symptomatic metastasis of an undifferentiated pleomorphic sarcoma of the lung to the right ventricle. She had a history of a stage IV, pulmonary, undifferentiated pleomorphic sarcoma that had been successfully treated with chemotherapy and radiotherapy 4 years ago. A complete response was obtained, and she was in remission until the cardiac metastasis. She underwent surgical excision of the cardiac mass because it caused dyspnea and posed a high risk of sudden death, pulmonary embolism or tricuspid obstruction. Histopathological and immunohistochemical examinations of the surgical specimen established the diagnosis of undifferentiated pleomorphic sarcoma and confirmed that the cardiac tumor was a metastasis from the lung.ConclusionsIn patients who have known metastatic neoplasms and present with cardiac manifestations, whether detected during history taking or physical examination, the clinician should be alert to the possibility of cardiac metastases. In patients with cardiac metastases, the therapeutic alternatives are limited to palliative treatment of symptoms and chemotherapy. In some patients, surgery can be used to relieve symptoms. We have reported the first case of symptomatic cardiac metastases from an undifferentiated pleomorphic sarcoma of the lung. Our patient underwent surgical resection, and her symptoms improved significantly. This case is unique because it is the only reported case of undifferentiated pleomorphic sarcoma of the lung which metastasized to the heart, and in which symptomatic improvement was effectively obtained with surgical resection.

Undifferentiated pleomorphic sarcoma with osteoclast-like giant cells of the female breast

The authors describe a case of undifferentiated pleomorphic sarcoma of the breast occurring in a 50-year-old woman who presented with a palpable mass in her right breast. She first noticed the mass one month previously. Core needle biopsy showed connective tissue including epithelioid and spindle cells. The patient underwent total mastectomy without axillary lymph node dissection. Based on examination of the excised tumor, the initial pathologic diagnosis was atypical spindle-shaped and ovoid cells with uncertain malignant potential. Histological findings with immunomarkers led to the final diagnosis of undifferentiated pleomorphic sarcoma.This case highlights a rare and interesting variant of primary breast sarcoma and the important role of immunohistochemistry in defining histological type and differential diagnosis. Hence, undifferentiated pleomorphic sarcoma has been a diagnosis of exclusion performed through sampling and critical use of ancillary diagnostic techniques.

肺癌精査中に発見された中咽頭未分化型多形肉腫の1例

肺癌に対する全身検索中に発見された中咽頭未分化型多形肉腫の1例を経験したので，報告する。症例は85歳，男性。右後口蓋弓から発生する境界明瞭，可動性良好，弾性硬で圧痛の伴わない腫瘤を認めた。全身麻酔下に腫瘍摘出術を施行し，未分化型多形肉腫の診断を得た。肺病変に対しては胸腔鏡下右上肺葉切除術を施行し，組織型は腺癌であった。高齢者であり認知症もあるため，術後加療は外来にてテガフール・ウラシル配合剤の内服治療を施行した。術後5か月目に肺癌由来と考えられる多発性脳転移が出現，ガンマナイフ治療を施行したが，1年目に再発，認知症も増悪，ADLの低下もきたし，積極的な治療は行えず，緩和治療目的に他院に転院。術後1年5か月目に転院先で転移性脳腫瘍のため永眠された。一般に，未分化型多形肉腫の加療は手術加療が優先され，文献的にも口腔，咽頭領域でも手術加療が治療の第一選択であると考えた。

A case of undifferentiated pleomorphic sarcoma of the stomach showing rapid growth

A case of undifferentiated pleomorphic sarcoma of the stomach showing rapid growth

Burn scar pleomorphic sarcoma—case report

Malignant transformation in burn scars was formally described by Jean-Nicholas Marjolin in 1828, although there are reports that this phenomenon had been first noted by Celsus in the first century A.D. The term Marjolin’s ulcer (MU) is used to denote the malignancies, predominantly squamous cell carcinomas, which develop in scars and chronic wounds [1–3]. The burned area, especially those which healed by secondary intention, is at risk of injury by daily activities because their coverage differs from normal skin, with reduced elasticity and hydration. The main factors that predispose to malignant transformation are: time of evolution, injury and constant irritation, chronic infection, poor hygiene habits, and environmental factors [1–3]. The percentage of scars that progress to malignancy is estimated at 2%, where squamous cell carcinoma is the most common histological type, followed by basal cell carcinoma and melanoma. Sarcomas, however, are rarely described [4–8]. The aim of this paper is to report a case of undifferentiated pleomorphic sarcoma in a burn scar treated at our service.