腹膜后促结缔组织增生性小圆细胞瘤临床病理研究(附一例报告及文献复习)

目的　探讨促结缔组织增生性小圆细胞瘤的临床病理特征。　方法　报告 1例 11岁促结缔组织增生性小圆细胞瘤患儿的诊治情况 ,复习有关文献。　结果　患儿以腹痛为主诉 ,左腹膜后 9.0cm× 6.0cm× 3 .0cm囊性肿瘤 ,细胞巢周围结缔组织显著增生 ,免疫组化染色CD99( )、EWS/WT1( )。腹膜后肿瘤切除术后联合化疗 ,随访 16个月无肿瘤复发。　结论　促结缔组织增生性小圆细胞瘤有特异的临床病理特征 ,好发于男性青少年。细胞巢周围结缔组织显著增生 ,表达MIC2基因产物CD99及特异的EWS/WT1蛋白 ,手术切除肿瘤联合化疗是治疗的主要方法。     

促纤维增生性小圆细胞肿瘤11例病理及免疫组化观察

目的探讨促纤维增生性小圆细胞肿瘤的临床病理特征及肿瘤与周围增生的纤维组织的关系。方法对11例促纤维增生性小圆细胞肿瘤进行HE光镜观察和免疫组化染色分析。结果肿瘤由巢团状小圆细胞组成，间质为分化程度不同的宽大的纤维结缔组织，与瘤组织界限清楚且无明显炎细胞浸润，免疫组化显示多向分化。结论促纤维增生性小圆细胞肿瘤的纤维结缔组织可能不属于反应性增生而属于肿瘤性增生，有待进一步做克隆分析研究证实。     

原发性睾丸附件恶性纤维细胞瘤1例报告并文献复习

目的:探讨原发性睾丸附件恶性纤维细胞瘤的临床表现、影像学特点、病理特征、治疗及预后。方法:报告1例原发性睾丸附件恶性纤维细胞瘤情况:患者男,年龄63岁。因"复发性左侧阴囊内无痛性肿物2年"求治。检查AFP为16ng/ml,β-HCG为0.44mIU/L。B超检查提示左侧阴囊内占位性病变(恶性肿瘤可能性大)。经左侧腹股沟切口行左侧阴囊探查+睾丸根治性切除术。结果:病理检查报告为(睾丸附件)恶性纤维组织细胞瘤,瘤组织未侵及睾丸,输精管切缘未见瘤细胞。免疫组织化学:CD68+,Desmin+++,Vimentin++,CK-,S-100-,Actin-。术后未行局部放疗及化疗。随访15个月,CT复查无局部肿瘤复发及远处转移表现,患者生存质量良好,目前仍在继续随访中。结论:原发性睾丸附件恶性纤维组织细胞瘤临床表现及影像学表现缺乏特征性,确诊依靠术后病理学检查,手术切除+术后辅助化疗为有效治疗方法。     

尿路移行上皮细胞癌睾丸转移一例报告

患者,84岁.左侧阴囊内肿块进行性增大4个月于2005年10月20日入院.查体:左阴囊内扪及2 cm×3 cm肿块,质地较硬.B超示左阴囊内低回声肿块1.24 cm×0.96 cm,与附睾分界不清,左睾丸受压变形.诊断:左侧睾丸旁肿块.硬膜外麻醉下行探查术,术中见左侧睾丸旁肿块,质硬,与睾丸粘连紧密.行左侧睾丸高位切除术.病理报告:左睾丸低分化癌,血管内见癌栓.     

促纤维增生性小圆细胞肿瘤4例报告

目的:探讨促纤维增生性小圆细胞肿瘤(DSRCT)的临床特点、诊断及治疗方法。方法:回顾性分析近5年间手术治疗4 例DSRCT患者的临床资料，诊断均经病理证实。结果:3例临床可扪及肿块，4例均有腹胀、消瘦，轻度贫血。4例通过B超、CT或MRI等辅助检查均发现肿瘤。3例为腹腔内肿瘤，1例胸腔内肿瘤。1例行肿瘤切除术， 2例行减瘤术治疗，1例行活检术。4例术后均经病理结合免疫组化检查确诊。结论:该病临床罕见,预后很差。手术治疗是根治的惟一方法。术后化疗以烷化剂和植物碱为主。     

腹腔促结缔组织增生性小圆细胞肿瘤4例临床分析

回顾性分析空军军医大学第二附属医院2014至2022年收治的4例腹腔促结缔组织增生性小圆细胞肿瘤患者的临床资料。2例放弃治疗, 随访4～9个月死亡。1例先后接受手术、化疗、靶向、免疫和中药抗肿瘤治疗, 随访12个月死亡。1例接受顺铂、多西他赛化疗并辅以立体定向放疗, 随访至今, 约27个月, 患者仍生存。本研究显示腹腔促结缔组织增生性小圆细胞肿瘤临床症状不典型且预后极差, 手术、化疗、放疗一体化的综合治疗方案可能提高患者的远期生存率。     

睾丸肿瘤治疗的探讨：附36例报告

通过对36例不同病理类型的睾丸肿瘤患者施以不同的治疗方法,从临床结果中得出如下结论:静脉联合化疗对精原细胞瘤效果极佳,可使腹腔型巨大原发灶明显缩小,以利切除;已有远处转移的晚期精原细胞瘤,手术前后施以CDDP为主的化疗可使转移灶消失,患者获得长期存活;睾丸肿瘤出现阴囊皮肤浸润,不论病理类型,作阴囊部分切除,均不影响预后;但有腹股沟皮肤浸润者,应禁忌手术。静脉联合化疗对非精原生殖细胞癌及其转移性肿块不敏感,术前应采取淋巴化疗,使原发灶缩小,转移灶消失,术后淋巴化疗可望代替传统的腹膜后淋巴结清除术。     

罕见睾丸纤维瘤/卵泡膜瘤1例报告

<正>1病例资料患者男,40岁,因右侧阴囊肿大17月余于2013年3月入院。查体双侧腹股沟区未见异常,右侧阴囊肿大明显,阴囊皮肤无异常,阴囊内扪及大小约10.5cm、质地较硬的实性包块,未扪及睾丸、附睾组织,压痛不明显,包块与周围组织分界尚清,肿块相连处及近侧精索柔软无压痛;左侧阴囊、睾丸附睾未见异常。肿瘤大体外观见图1。阴囊彩超提示:右侧睾丸肿大回声不均,考虑畸胎瘤?其他良性肿瘤待排,左侧附睾头囊肿。盆腔MRI结果:右侧睾丸占位性病变,考虑精原细胞瘤可能,左侧睾丸鞘膜积液;全腹部CT平扫+增强:未见腹腔及盆腔淋巴结转移,右侧腹股沟区淋巴结稍大,约8mm,未见粘连;睾丸肿块考虑生殖细胞肿瘤,精原细胞瘤可能。生化     

睾丸鞘膜孤立性纤维假瘤1例报告并文献复习

目的探讨睾丸鞘膜孤立性纤维假瘤的临床病理学特点。方法回顾性分析1例睾丸鞘膜孤立性纤维假瘤患者的临床及病理资料,并进行文献复习。结果经阴囊切口探查为睾丸鞘膜孤立、光滑的肿块,并行肿瘤及部分睾丸鞘膜切除。术后病理报告为睾丸鞘膜纤维性假瘤。随访2年未见肿瘤复发及转移。结论睾丸鞘膜孤立性纤维假瘤罕见,应与该部位相似形态的病变和血管外皮瘤样的肿瘤相鉴别。     

精索多形性脂肪肉瘤1例报告并文献复习

目的：分析并总结精索脂肪肉瘤患者的临床表现及其诊断及治疗方法。方法：回顾性分析我院收治的1例精索脂肪肉瘤患者的临床资料：左侧阴囊内有-9．0cm×9．4cm×6．7cm肿块,B超检查示左侧阴囊内存在一非均质实性包块;MRI显示左侧阴囊内有实质性肿瘤,未见肿大淋巴结。血清肿瘤标志物13-HCG、CEA、PSA和AFP检查均无异常。手术切除肿瘤的同时行左侧阴囊部分切除术。结果：术后病理检查提示为多形性混合型脂肪肉瘤。患者术后恢复顺利。结论：精索脂肪肉瘤是一种罕见的泌尿生殖系肿瘤,诊断主要依据病理检查结果确定;治疗以根治性睾丸切除术为主要方式,辅助放疗、化疗疗效不确切;预后与肿瘤组织病理类型密切相关,局部复发很常见,需长期随访。     

Paratesticular desmoplastic small round cell tumor: case report

BACKGROUND: The desmoplastic small round cell tumor has recently been separated from other small round cell tumors because of its characteristic pathological and clinical features. They are usually intra-abdominal tumors affecting young people and have classically been associated with a bad prognosis. However, in recent years there have reports on desmoplastic small round cell tumors affecting other body regions, including the paratesticular area. CASE PRESENTATION: We report the case of a 23-year-old male, that consulted on a progressive enlargement of the right hemiscrotum in the last year and a half. He referred no previous urological symptoms and had no systemic symptomatology. Physical examination revealed a round elastic firm 2 to 3 cm mass distal to the tail of the epididymis, which was excised with a preoperative diagnosis of adenomatoid tumor. However, histological and immunohistochemical diagnosis confirmed a desmoplastic small round cell tumor. The extension study included a computed tomography scan and a plain chest radiograph, that showed no metastasis. The patient received chemoradiation therapy with methotrexate, dacarbacin, cyclophosphamide, actinomycin D and vincristin, but had to be changed to a vincristin, actinomycin D, cyclophosphamide and adriamicin scheme on severe toxicity. He completed five cycles of the chemotherapy with moderate toxicity. Today, 6 years after diagnosis the patient remains well and free of disease. CONCLUSIONS: Recent reviews on desmoplastic small round cell tumor affecting the paratesticular area have shown a better prognosis for tumors of this origin compared to abdominal ones. We should include this lesion among the differential diagnosis of paratesticular tumors, mainly in children and adolescents.     

Combined resection and multi-agent adjuvant chemotherapy for intra-abdominal desmoplastic small round cell tumour: case report and review of the literature

Desmoplastic small round cell tumor (DSRCT) is a rare, highly aggressive malignancy with distinctive histological and immunohistochemical features occurring in young population with male predominance. We report a case of DRSCT occurred in a 17 years old patient which presented with a large upper left quadrant abdominal mass that was treated with a very aggressive surgical approach and multi-agent chemotherapy. At a 12 months follow-up he is free of recurrence. This kind of tumour has a very poor prognosis. No standard treatment protocol has been established. Aggressive surgery combined with postoperative multi-agent adjuvant chemotherapy is justified not only to relieve symptoms but also to try to improve the outcome.     

Primary paratesticular epithelioid leiomyosarcoma

We report a case of a 69-year-old man with a history of left spermatic cord thickening and an enlarging painless mass in the left hemiscrotum. A final diagnosis of paratesticular epithelioid leiomyosarcoma was made. 13 months after treatment he is alive without any signs of tumor recurrence or metastasis.     

Fibrous pseudotumor of tunica vaginalis and epididymis

Paratesticular fibrous pseudotumors are rare, and less than 10% of them affect the epididymis. We report a case of testicular trauma that progressed to a painless palpable tumor in the right hemiscrotum with increased local volume. Orchiectomy followed by anatomic-pathologic examination revealed a paratesticular fibrous pseudotumor. The best treatment is scrotal exploration and frozen biopsy. On confirmation of the diagnosis, only the tumor and the tunica vaginalis are resected. Should the benign nature not be possible to determine, orchiectomy is the procedure of choice. We also reviewed published reports for this possibility in the differential diagnosis of testicular masses.     

Testicular and paratesticular neoplasms in prepubertal males

PURPOSE: We reviewed the current diagnosis, staging and management of testicular and paratesticular neoplasms in prepubertal males. MATERIALS AND METHODS: We performed a medical literature search in English using MEDLINE/PubMed that addressed testicular and/or paratesticular neoplasms in prepubertal males. We then analyzed the literature with respect to individual tumors. We present a concise approach toward the management of these individual tumors. RESULTS: There is still a predominance of yolk sac tumors in prepubertal males, although some studies suggest that teratomas are more common but underreported due to their benign course in children. Prepubertal males are diagnosed in a fashion similar to that in adult patients with an appropriate history and physical examination. A palpable, nontender mass suggests the diagnosis and prompts scrotal ultrasound and tumor markers. Although treatment for most primary tumors has historically been radical inguinal orchiectomy, most benign tumors can now be managed by testis sparing surgery. The addition of radiation, chemotherapy and/or retroperitoneal lymph node dissection depends on tumor stage and histological type. CONCLUSIONS: Although it is rare in children, any solid scrotal mass in prepubertal males warrants evaluation for possible testicular or paratesticular neoplasm.     

Burned-out testicular tumor: a case report

A 29-year-old man was referred to our hospital with loss of appetite and a left lower abdominal mass. A small nodule was palpable in his left testis and ultrasonographic examination demonstrated that the nodule was low echoic. Computed tomography showed a large mass in his left retroperitoneal space. We thought the mass was a metastatic lesion from a testicular tumor. Left orchiectomy was done and microscopic examination revealed no viable tumor cells. Only fibrous tissue, small calcified areas, and hyaline bodies were found. As tumor markers were normalized after 3 courses of chemotherapy with bleomycin, etoposide, and cisplatine, the retroperitoneal mass was removed with the left kidney. It consisted of embryonal carcinoma, mature teratoma, and yolk sac tumor. One course of adjuvant chemotherapy was done and the patient has since been free from recurrence. We suppose that the tumor was a so-called 'burned-out' testicular tumor.     

Cystadénome Papillaire de L’épididyme: Un Nouveau Cas

Papillary cystadenoma of the epididymis is a rare benign neoplasm that arises from the efferent duct epithelium. It may show as a unilateral or bilateral epididymal mass and its association with von Hippel-Lindau syndrome is very high, particularly in bilateral lesions. A 36-year-old male presented with a left-side scrotal mass of one year duration. On local examination, a painless irregular and hard testicular mass. Ultrasonography of the scrotum revealed a left strong hypoechoic, homogeneous, well limited testicular mass of 30×25×22mm diameter. The tumorous scorers were normal (beta HCG and AFP). The testicular mass was explored through a left inguinal incision. In palpation, it is a hard testicular tumor. Left Orchidectomy is carried out. The pathologic examination reveals a serous papillary cystadenoma borderline paratesticular without sign of invasion. We propose guidelines from literature for diagnosis, histogenesis and treatment of this rare tumor.     

Polyorchidim: a case report and review of the literature

A 28-year-old male presented with a small painless lump in his left hemiscrotum. A physical examination revealed a non-tender mass that was palpable on the tail of left epididymis, and the testis and spermatic cord were normal. Ultrasonography showed an isoechoic round shaped tumor, 16 mm in diameter. An exploration of the scrotum was performed, based on a preoperative diagnosis of a left epididymal tumor. The tumor was located below the tail of epididymis, and had a whitish capsule, which looked similar to tunica albuginea testis. A frozen section revealed testicular tissue without any malignant change, and therefore polyorchidism was diagnosed. The accessory testis was resected because there was no connection with the epididymis and vas deferens. Polyorchidism is a rare congenital anomaly with 24 cases reported in the Japanese literature. The indications for the resection of an accessory testis are controversial. Patients with intrascrotal polyorchidism might be recommended to undergo a resection of the accessory testes if there are signs of dysplasia during an intraoperative biopsy. Patients must be followed up with regular clinical and ultrasonic examinations when accessory testes are preserved. However, extrascrotal supernumerary testes should be managed by an orchiectomy because of the increased risk of malignancy.     

A case of bilateral testicular tumors showing remarkable regression of huge metastatic tumors after VAB-6 combined chemotherapy

A case of bilateral testicular seminomas with abdominal huge metastatic tumors is presented. The patient is a 23-year-old male. An abdominal huge mass was found incidentally by a physician. CT scan and ultrasonography revealed the presence of the tumor in the left retroperitoneal space and biopsy specimen of the abdominal tumor was diagnosed as seminoma. On March 7, 1985, he was referred to our clinic. Bilateral testicular tumors were detected on palpation and ultrasonography. Bilateral orchiectomy was performed. Histological diagnosis was pure seminoma. After four sessions of VAB-6 combined chemotherapy, the abdominal tumor, 14.1 x 12.3 cm in size, decreased to 5.7 x 4.4 cm ( a regression rate of 85.5%). Retroperitoneal lymph-node dissection was undertaken, but the abdominal tumor could not be resected completely. Histological examination of the resected tumor revealed complete necrosis of the tumor tissue. After the operation, one session of the chemotherapy and irradiation were added. A total of 109 cases of bilateral testicular germ cell tumors in Japan was reviewed.