Chiloleti Geofrey, Lituli Hashim, Sobbo Salim, Sensa Victor, Mushi Fransia
Department of Surgery, School of Medicine, Muhimbili University of Health and Allied Sciences, Dar es Salaam, Tanzania.
Department of Urology, Muhimbili National Hospital, Dar es salaam, Tanzania.
Int J Surg Case Rep. 2024 Oct;123:110296. doi: 10.1016/j.ijscr.2024.110296. Epub 2024 Sep 17.
Rhabdomyosarcoma [RMS] is a malignant soft-tissue neoplasm characterized by skeletal muscle differentiation. It accounts for 7 % of childhood malignancies and is, by a wide margin, the most common sarcoma of childhood (Pappo, 1996). Approximately 20 % of cases of childhood rhabdomyosarcoma occur in the genitourinary tract (vagina, urinary bladder, prostate, paratestis, and uterus), and they are most commonly observed in the head and neck region.
We reviewed the case of a 42-year-old male who presented with a 2-week history of blood in the urine, which was painless, started on gradual onset, and progressively increased, involving the whole stream of urine. The blood clots were amorphous in shape but had no history of passed tissue shreds, childhood schistosomiasis, cigarette smoking, or working in chemical industries. On physical examination, the patient was anxious and conscious, with normal vital signs. The abdomen revealed distended abdomen shifting dullness, a palpable urinary bladder with suprapubic distension, and a tenderness on palpation, which disappeared upon catheterization 3-way 24F, with normal male genitalia and right lower limb edema. Other systems were essentially normal. On image KUB USS revealed a bladder mass, Cystoscopy showed a broad base bleeding tumor located at dome to the left lateral, fungating, with some necrotic tissue. TURBT was done into completion and histology revealed an embryonal rhabdomyosarcoma of urinary bladder, a botryoid subtype. After TURBT, the patient received adjuvant chemotherapy, the MAID protocol and underwent a 6-cycle cycle. The cycle was repeated every 21 days, and his height was 171.5 c, and his weight was 89 kg. Serial of check cystoscopy for one year revealed no recurrency of tumor. He repeated check CT scan, which showed a radiological improvement compared to the initial image.
Rhabdomyosarcoma in adults is a rare type of urinary bladder carcinoma that is quite aggressive and is usually reported to be a pediatric malignant urinary bladder tumor. The modality of treatment is not universal because of its rarity. We used a combination of TURB and chemotherapy and performed strict follow-up, with no tumor recurrence occurring at least after one year of follow-up. These patients show significant improvement from the first presentation, both clinically and radiologically.
The lack of a universal standard treatment approach for adult rhabdomyosarcoma indicates the need for more data on adult rhabdomyosarcoma, with a detailed description of its histological subtype.
横纹肌肉瘤[RMS]是一种以骨骼肌分化为特征的恶性软组织肿瘤。它占儿童恶性肿瘤的7%，是儿童期最常见的肉瘤（帕波，1996年）。儿童横纹肌肉瘤约20%发生于泌尿生殖道（阴道、膀胱、前列腺、睾丸旁组织和子宫），最常见于头颈部区域。
我们回顾了一例42岁男性病例，该患者有2周无痛血尿病史，起病隐匿且逐渐加重，累及全程尿液。血凝块形状不规则，但无组织碎片排出史、儿童血吸虫病史、吸烟史或化工行业工作史。体格检查时，患者焦虑但意识清醒，生命体征正常。腹部检查发现腹部膨隆、移动性浊音阳性、可触及膀胱且耻骨上区膨隆，触诊时有压痛，插入24F三腔导尿管后压痛消失，男性生殖器正常，右下肢水肿。其他系统基本正常。腹部平片及超声检查显示膀胱占位，膀胱镜检查发现一个位于膀胱顶部至左侧壁的广基出血性肿瘤，呈蕈伞状，伴有一些坏死组织。经尿道膀胱肿瘤切除术（TURBT）完整切除肿瘤，组织学检查显示为膀胱胚胎型横纹肌肉瘤，葡萄状亚型。TURBT术后，患者接受了辅助化疗，采用MAID方案，共进行6个周期，每21天重复一个周期，患者身高171.5厘米，体重89千克。术后一年的系列膀胱镜检查未发现肿瘤复发。复查CT扫描显示与初始影像相比有影像学改善。
成人横纹肌肉瘤是一种罕见的膀胱癌类型，侵袭性很强，通常被认为是儿童期恶性膀胱肿瘤。由于其罕见性，治疗方式并不统一。我们采用了TURBT和化疗相结合的方法，并进行了严格的随访，至少随访一年未发现肿瘤复发。这些患者从首次就诊时起，临床和影像学表现均有显著改善。
成人横纹肌肉瘤缺乏统一的标准治疗方法，这表明需要更多关于成人横纹肌肉瘤的数据，并对其组织学亚型进行详细描述。
