PDF(Pubmed)
Abstract：
UNASSIGNED: Malignant triton tumors (MTT) are subtype of malignant peripheral nerve sheath tumor (MPNST) which develop from Schwan cells of peripheral nerves or within neurofibromas, and shows rhabdomyoblastic differentiation. It is a rare soft tissue tumor with poor prognosis.
UNASSIGNED: We report a case of Malignant Triton Tumor (MTT) arising in the right shoulder in a 46 year old male patient presented to our Musculoskeletal Oncology Clinic at Royal Rehabilitation center at King Hussein Medical Center during June 2018.
UNASSIGNED: The patient was complaining of an 8 months long progressive right shoulder pain and swelling at the posterior lateral area of the shoulder. As accurate diagnosis is crucial in such case, investigations that included x-rays and magnetic resonance imaging (MRI) demonstrated an soft tissue tumor involving the right shoulder area leading to the differential diagnosis of aggressive soft tissue tumor which laid down the plan of an open incisional biopsy to be reported histopathological as a case of Malignant Triton Tumor which is a very rare and aggressive sarcoma originates from the peripheral nerve sheaths as it is subtype of malignant peripheral nerve sheath tumors after which excision of the entire tumor with safety margin was performed and referred for adjuvant chemotherapy.
UNASSIGNED: The treatment of choice is radical tumor excision with wide margins followed by chemotherapy and /or radiotherapy to improve the 5 years survival rates.
UNASSIGNED: We report a case of Malignant Triton Tumor (MTT) arising in the right shoulder in a 46 year old male patient presented to our Musculoskeletal Oncology Clinic at Royal Rehabilitation center at King Hussein Medical Center during June 2018.
UNASSIGNED: The patient was complaining of an 8 months long progressive right shoulder pain and swelling at the posterior lateral area of the shoulder. As accurate diagnosis is crucial in such case, investigations that included x-rays and magnetic resonance imaging (MRI) demonstrated an soft tissue tumor involving the right shoulder area leading to the differential diagnosis of aggressive soft tissue tumor which laid down the plan of an open incisional biopsy to be reported histopathological as a case of Malignant Triton Tumor which is a very rare and aggressive sarcoma originates from the peripheral nerve sheaths as it is subtype of malignant peripheral nerve sheath tumors after which excision of the entire tumor with safety margin was performed and referred for adjuvant chemotherapy.
UNASSIGNED: The treatment of choice is radical tumor excision with wide margins followed by chemotherapy and /or radiotherapy to improve the 5 years survival rates.
摘要：
■恶性triton肿瘤（MTT）是恶性周围神经鞘瘤（MPNST）的亚型，由周围神经或神经纤维瘤的Schwan细胞发展而来，并显示横纹肌母细胞分化。是一种罕见的软组织肿瘤，预后较差。
我们报告了一名46岁男性患者的右肩恶性Triton肿瘤（MTT）病例，该患者于2018年6月在侯赛因国王医疗中心皇家康复中心的肌肉骨骼肿瘤诊所就诊。
患者主诉8个月的进行性右肩疼痛和肩后外侧区肿胀。在这种情况下，准确的诊断至关重要，包括X线和磁共振成像（MRI）在内的研究表明，软组织肿瘤累及右肩区域，从而对侵袭性软组织肿瘤进行了鉴别诊断，并制定了开放切开活检的计划，以组织病理学报告为一例。恶性Triton肿瘤是一种非常罕见且侵袭性的肉瘤，起源于周围神经鞘，因为它是恶性周围神经鞘肿瘤的亚型，此后对整个肿瘤进行了辅助切除，并进行了安全化疗。
■选择的治疗方法是广泛的肿瘤切除，然后进行化疗和/或放疗，以提高5年生存率。
我们报告了一名46岁男性患者的右肩恶性Triton肿瘤（MTT）病例，该患者于2018年6月在侯赛因国王医疗中心皇家康复中心的肌肉骨骼肿瘤诊所就诊。
患者主诉8个月的进行性右肩疼痛和肩后外侧区肿胀。在这种情况下，准确的诊断至关重要，包括X线和磁共振成像（MRI）在内的研究表明，软组织肿瘤累及右肩区域，从而对侵袭性软组织肿瘤进行了鉴别诊断，并制定了开放切开活检的计划，以组织病理学报告为一例。恶性Triton肿瘤是一种非常罕见且侵袭性的肉瘤，起源于周围神经鞘，因为它是恶性周围神经鞘肿瘤的亚型，此后对整个肿瘤进行了辅助切除，并进行了安全化疗。
■选择的治疗方法是广泛的肿瘤切除，然后进行化疗和/或放疗，以提高5年生存率。
