Koppisetty Shalini, Alessio Ricardo C, Rajpurkar Atul
Department of Infectious Diseases, Beaumont Hospital, Royal Oak, MI, USA.
Department of Pathology, Crittenton Hospital Medical Center, Rochester, MI, USA.
Urol Ann. 2016 Jul-Sep;8(3):387-90. doi: 10.4103/0974-7796.184878.
Malignant peripheral nerve sheath tumors (MPNSTs) are extremely rare soft tissue sarcomas of ectomesenchymal origin. They are commonly seen in association with neurofibromatosis type 1 (NF-1), but can also occur without a history of NF (isolated MPNST). MPNSTs are most commonly located on the extremities (brachial and sacral plexus), head and neck, and trunk regions and are rarely reported in genitourinary organs. These tumors are aggressive, with a high recurrence rate and distant metastases. MPNST involving the kidney is extremely rare, and review of the literature using PubMed from 2001 to 2014 revealed eight cases of MPNST involving the kidney (seven, primarily involving the kidney and one metastatic MPNST of the kidney). Herein, we describe a case of breast metastases from an MPNST of the kidney without a history of NF-1. The patient was initially diagnosed with a spindle cell neoplasm of the kidney with peripheral nerve sheath differentiation. Eventually, the patient developed a right breast mass that was diagnosed as metastatic MPNST. The patient refused any kind of treatment and died 6 months later in hospice care.
恶性外周神经鞘瘤(MPNSTs)是起源于外胚间叶组织的极为罕见的软组织肉瘤。它们通常与1型神经纤维瘤病(NF-1)相关,但也可在无NF病史的情况下发生(孤立性MPNST)。MPNST最常见于四肢(臂丛和骶丛)、头颈部及躯干区域,很少报道发生于泌尿生殖器官。这些肿瘤具有侵袭性,复发率高且易发生远处转移。累及肾脏的MPNST极为罕见,使用PubMed对2001年至2014年的文献进行回顾,发现有8例累及肾脏的MPNST(7例主要累及肾脏,1例为肾脏转移性MPNST)。在此,我们描述1例无NF-1病史的肾脏MPNST发生乳腺转移的病例。该患者最初被诊断为具有外周神经鞘分化的肾脏梭形细胞瘤。最终,患者出现右侧乳腺肿块,被诊断为转移性MPNST。患者拒绝任何治疗,6个月后在临终关怀中死亡。