Sex cord-stromal tumors of the testis with entrapped germ cells: a lesion mimicking unclassified mixed germ cell sex cord-stromal tumors

The authors describe 10 sex cord-stromal tumors of the testis that incorporated germ cells, thereby mimicking the unclassified type of mixed germ cell sex cord-stromal tumor (MGCSCST). These neoplasms occurred in patients from 3 to 48 years old (mean age, 26 years) who presented with testicular masses. On microscopic examination, nine tumors had a combination of tubular and cord-like arrangements of sex cord cells with transition to spindle-shaped tumor cells. They were diagnosed as either unclassified sex cord-stromal tumors (n = 5) or Sertoli-stromal cell tumors (n = 4). One tumor was a pure Sertoli cell tumor. The admixed germ cells were usually at the periphery and in clusters, but occasionally were in the center or more diffuse. In nine patients the germ cells resembled spermatogonia, having round nuclei with uniform, dusty chromatin and inconspicuous or small nucleoli. None of these cells stained with a variety of markers used for neoplastic germ cells, and in one case in which the non-neoplastic Sertoli cells were strongly reactive for inhibin but the neoplastic Sertoli cells were not, all the germ cells within the tumor occurred adjacent to inhibin-positive Sertoli cells. With static cytophotometry, a diploid deoxyribonucleic acid content was found in these germ cells in the two investigated cases. In one case the germ cells had the morphologic appearance of seminoma cells and they stained positively for the markers of neoplastic germ cells. This case was interpreted as a "collision" tumor between a Sertoli cell tumor and a seminoma. The authors conclude that sex cord-stromal tumors with entrapped germ cells of the testis are more common than unclassified MGCSCSTs--a bona fide testicular example of which has not been seen by any of the authors.     

The spectrum of Sertoli cell tumors in children

The spectrum of Sertoli cell tumors in children covers a wide range of testis and ovarian tumors classified as sex cord-stromal tumors. Sertoli cell tumor of the testis is extremely rare in the pediatric population. The American Academy of Pediatrics Section on Urology Prepubertal Testicular Tumor Registry has reported a total of six cases of Sertoli cell tumor of the testis, accounting for 1.3% of the 430 cases reported to the registry as of October 1996. Despite their rarity, Sertoli cell variants of sex cord-stromal tumors have generated keen interest because of their variable histologic appearance and biologic behavior, including endocrine activity. Because sex cord-stromal tumors occur in the ovary and testis, a primitive cellular origin to these tumors is likely in males and females.     

Sclerosing Sertoli cell tumor of the testis. A report of 10 cases.

Sex cord-stromal tumors of the testis are uncommon and have been less well characterized than similar tumors of the ovary, with a much greater proportion of them falling into the "unclassified" category. We report the clinical and pathological features of 10 Sertoli cell tumors of the testis with prominent sclerosis, representing a distinctive, heretofore undescribed subtype of Sertoli cell tumor in the human. The patients were mostly in their 3rd and 4th decades (median age, 30 years; range, 18-80 years). One tumor occurred in a cryptorchid testis, and one patient had had an orchidopexy several years prior to his presentation. There was no evidence of estrogen production by the tumor in any case. The tumors occurred equally often in each testis and were small (0.4-1.5 cm) in diameter, except for two tumors, which were 4.0 cm in diameter. All of them were centered in the testicular parenchyma and were well-demarcated, hard, yellow-white to tan nodules. They were characterized histologically by solid and hollow, simple and anastamosing tubules, large irregular aggregates, and thin cords of Sertoli cells in a prominent collagenous background. The tumor cells were of medium size and had pale cytoplasm, which sometimes contained large lipid vacuoles; the round nuclei varied from small and dark to large and vesicular. Follow-up information on five patients--including the only one whose tumor had malignant features histologically--showed no evidence of recurrence or metastasis 3-10 years (mean, 5.8 years) after orchidectomy alone.     

Tiny nodule in the testicle: Case report of a sertoli cell tumor

Sex cord-stromal tumors of the testis are rare. We report on a small Sertoli cell tumor in the testicle. According to published reports, a nodular lesion on the testicle has a variety of differential diagnoses. Preoperatively, it is very difficult to differentiate between a tumorous lesion and an inflammatory change. When a tiny nodule in the testicle is encountered, we propose limited, testicular-sparing surgery according to the frozen section diagnosis.     

Malignant undifferentiated sex cord-stromal testis tumor with brain metastasis: case report

Metastatic undifferentiated sex cord-stromal testis tumors are uncommon. We would like to present a rare case of undifferentiated sex cord-stromal testis tumor with brain metastasis. After presenting with a painless right testicular mass, the patient underwent right radical orchiectomy and retroperitoneal lymph node dissection. One month later, the patient had no visible evidence of tumor recurrence on the follow-up computed tomography of the chest, abdomen, and pelvis. Three months after the first follow-up, the patient was readmitted for new onset of shortness of breath, and 7th and 12th cranial nerve palsy. Computed tomography of the chest and magnetic resonance imaging of the brain showed evidence of distant metastasis. To our knowledge, undifferentiated sex cord-stromal tumor with brain metastasis has not been reported. As with any new onset neurologic deficits in patients with solid tumors, the presence of brain metastases should be considered.     

胃低分化腺癌合并印戒细胞癌睾丸转移1例报告并文献复习

目的:探讨我院1例罕见胃印戒细胞癌睾丸转移的临床病理特点,免疫组织化学在睾丸转移瘤与原发肿瘤鉴别诊断中的应用。方法:回顾分析了我院收治1例睾丸肿瘤患者,1年前曾行胃贲门癌根治性全胃切除术,术后病理报告提示低分化印戒细胞癌,术后未予以放化疗,此次外院睾丸穿刺活检病理报告为低分化腺癌浸润或转移,考虑患者的病情及预后,行患侧根治性睾丸切除术。结果:术中见睾丸略增大,约4cm×3cm×2cm,质地硬,颜色呈淡红色,附睾大小、质地无异常。术后病理证实胃低分化腺癌合并印戒细胞癌转移。随访半年,临床症状好转。结论:胃印戒细胞癌睾丸转移非常罕见,特定的免疫组织化学指标可以有助于诊断。     

Conservative management of bilateral Sertoli cell tumors of the testicle in association with the Carney complex: a case report

Large cell calcifying Sertoli cell tumor of the testicle is a rare, hormonally active sex cord-stromal tumor seen in patients with Carney complex. When such tumors occur bilaterally, treatment options for preserving fertility and addressing the secondary effects of excess hormone production must be considered. The availability of specific antiestrogen drugs means that bilateral orchiectomy for this benign tumor may no longer be warranted. Testicular-sparing surgery and advances in reproductive technology may also improve the overall prognosis for fertility.Gynecomastia in prepubescent boys can be emotionally very distressing. Approximately two thirds of teenaged boys will develop some degree of breast enlargement that spontaneously regresses as testosterone levels rise (Ill Med J 1938;73:113). In all cases, a thorough history and physical examination are required to exclude nonphysiologic causes such as drugs, pulmonary disease, chronic liver disease, exogenous estrogens, and estrogen-producing tumors (Seashore J. Disorders of the breast. In: Rowe MI, O'Neill JA, Grosfeld JL et al, editors. Pediatric surgery, 5th ed. St Louis (MO): Mosby Year Book, 1998).We report on a child who presented with a 2-year history of gynecomastia with associated bilateral testicular swellings and discuss a novel treatment strategy for managing bilateral testicular tumors in the context of the Carney complex.     

A case of testicular angiomyolipoma

Abstract:   The majority of testicular tumors are germ cell tumors, which are the most prevalent solid malignancies in young adult males. Non-germ cell tumors of the testis are rare. Here, we report a case of testicular angiomyolipoma (AML). A 22-year-old male underwent left orchiectomy under a diagnosis of testicular tumor. Pathological analysis demonstrated that it was composed of adipose tissue and vasculature with foci of myomatous component. The tumor demonstrated neither cytological atypia nor widespread mitotic activity. In addition, the tumor cells showed intense expression of CD34 and smooth muscle actin, whereas HMB-45 was entirely negative. Although the true cellular origin and its clinical implications remain unknown, pathological and immunohistochemical studies strongly indicated benign testicular AML with a non-germ cell origin. To our knowledge, this is only the second case of a primary testicular AML in the published reports and the detailed pathological findings are first described in this report.     

Undifferentiated Sex Cord—Stromal Tumor in a Young Adult

Undifferentiated sex cord-stromal tumor in post-puberty male is extremely rare. There were only three reported cases in the literature. We reported a 19-year-old patient presented with an asymptomatic right testicular nodule with normal level of serum marker for germ cell tumor. Excisional biopsy and subsequent orchidectomy was preformed and the final pathology supported the diagnosis of undifferentiated sex cord-stromal tumor. He was then put on regular surveillance with no adjuvant therapy given. He remained disease free 18 months after the operation. A summary of the literatures and discussion on the management of this rare tumor was provided.     

Testicular carcinoid tumor: A case report and literature review

We present a case of a 46-year-old man with an incidental finding of a right testicular tumor on magnetic resonance imaging. He received radical orchiectomy, and the pathological report showed a testicular carcinoid tumor. He has remained in a stable condition 3 years after the operation. Carcinoid tumors of the testis are extremely rare and account for less than 1% of testicular tumors and carcinoids. A painless mass or prominent testis enlargement is the most distinctive presentation. Less than 1–3% of patients demonstrate carcinoid syndrome. Nuclear scintigraphy scan and computed tomography scan have a diagnostic value in excluding carcinoid from other sites. Radical orchiectomy is the treatment of choice, and the prognosis is good for localized carcinoid tumors after resection. Adjuvant chemotherapy or radiotherapy for advanced disease shows only minimal benefits.     

Bilateral primary germ cell tumors of the testis: report of two cases and review of the literature

Bilateral germ cell tumors of the testis are not common. The contralateral tumor may develop simultaneously or sequentially. In both cases, bilateral involvement is postulated to represent two primary tumors. Case 1: A 47-year-old male was seen with a painless nodular mass in the right testis. Physical examination revealed hard swelling of both testes besides a nodule in the right testis. He underwent bilateral radical orchiectomy; and, microscopical both tumors proved to be seminoma. Although bipedal lymphangiography was negative for nodal metastases, postoperative irradiation to the pelvic and para-aortic lymph nodes was given to a total dose of 5,800 rad. The patient was alive 15 years after treatment for bilateral testicular tumors. Case 2: A 53-year-old male presented with painless left scrotal swelling. Physical examination showed not only an enlarged hard testis in the left scrotum but also a palpable hard mass in the right testis. Bilateral radical orchiectomy was performed and pathological examination revealed pure seminoma in both testes; a 1 cm mass in the right testis and almost completely replaced tumor in the left testis. Evaluation including retroperitoneal lymph node dissection revealed no metastatic disease. Postoperatively, the patient received 1,900 rad of irradiation to the retroperitoneal space. He is well one year after surgery and has no evidence of recurrence or metastasis. Previous reports of bilateral germ cell testicular tumors are reviewed and the incidence, age, predisposing factors, interval between the first and second tumors, histology, pathogenesis, prognosis, and management are discussed.     

睾丸原发性类癌的临床病理学观察

目的:探讨原发于睾丸的类癌临床病理学特征、免疫表型、鉴别诊断及其组织起源。方法:应用光镜、免疫组织化学对4例原发于睾丸的类癌进行检测。结果:4例患者均表现为阴囊肿块,病程2～36年,其中2例伴有会阴胀痛感。肿块呈结节状,直径3.0～4.0cm,切面灰黄色,界清,1例有灶性坏死。组织学上,癌细胞呈圆形、多角形,胞质略嗜伊红颗粒状,胞界不清。核单一,圆形,染色质点彩状。癌细胞排列呈岛状、梁状,可见小的规则的菊形团样结构及大的不规则腺管样结构,腺腔内可见红染的分泌物。癌巢间为纤细的纤维组织间隔。免疫表型,癌细胞弥漫性表达突触素、嗜铬素、神经元特异性烯醇酶和细胞角蛋白。结论:睾丸原发性类癌罕见,组织起源有争议。该肿瘤应与精原细胞瘤、转移性类癌、支持细胞瘤及粒层细胞瘤等鉴别。     

Classification and pathology of testicular germ cell and sex cord-stromal tumors.

Germ cell tumors of the testis are the most frequent testicular neoplasms, with seminoma predominating. The pathologist must be able to discriminate between seminoma and nonseminomatous germ cell tumors as well as sex cord-stromal tumors and metastatic lesions. Appropriate therapy and accurate prognostic information are dependent on the proper classification of testicular neoplasia. Characteristic histologic features, serum markers, and immunohistochemistry are helpful in this regard. Sex cord-stromal tumors comprise a small minority of testicular neoplasms. It remains critically important not to confuse these neoplasms with testicular germ cell or metastatic tumors, and, again, recognition of the characteristic histologic features, immunohistochemical findings, and clinical information is diagnostic. The urologist can provide the pathologist with key clinical information in the attempt to make a correct diagnosis.     

Juvenile granulosa cell tumor of the testis:: contemporary clinical management and pathological diagnosis

PURPOSE: Juvenile granulosa cell tumor (JGCT) of the testis is a rarely diagnosed subset of testicular stromal tumors. Although this variant of testicular stromal tumor is predominantly a benign entity in prepubertal patients, limited experience precludes a complete understanding of its clinical presentation and pathological diagnosis. MATERIALS AND METHODS: We reviewed all cases of testicular tumors at Children's Hospital of Philadelphia between 1976 and 2002 in males younger than 18 years. We specifically reviewed our experience with JGCT in terms of presentation, surgical treatment and long-term outcome. We also reviewed the microscopic findings and histochemical techniques used to confirm the diagnosis. RESULTS: We identified 77 tumors during the defined interval, of which 3 (3.9%) were JGCTs. All 3 patients with JGCT were first noted to have a testis mass soon after birth. All presented with a firm, unilateral testicular mass. Ultrasonographic findings were consistent with a complex, multiseptated, hypoechoic mass. Two of the 3 patients underwent radical orchiectomy. Testis sparing mass excision was performed in 1 patient. Grossly the tumors were partially cystic masses. Histologically positive immunostaining with inhibin-alpha and negative staining for alpha-fetoprotein (AFP) reliably differentiated JGCTs from yolk sac tumors. At a mean followup of 8.5 years (range 5 to 14) no metastases or local tumor recurrences have been diagnosed. CONCLUSIONS: To our knowledge we report the first case of testis sparing enucleation of a JGCT with a 5-year recurrence-free followup. Testis sparing enucleation is now our procedure of choice for tumors in neonates and prepubertal children with serum AFP in the normal range for age. JGCT should be suspected in neonates presenting at birth with a complex, cystic mass of the testis. Positive immunostaining for inhibin-alpha and a lack of AFP staining have consistently corroborated the pathological diagnosis in our experience and they should be applied for pediatric testis tumors that may mimic yolk sac tumor pathology.     

睾丸原发性神经内分泌癌1例报告并文献复习

目的：探讨睾丸原发性神经内分泌癌的起源、诊断要点和治疗方法,提高对该疾病的认识。方法：回顾性分析我院收治的1例睾丸原发性神经内分泌癌患者的资料,并复习有关文献。结果：患者左侧睾丸有一无痛肿块,质硬。彩超扫描证实左侧睾丸内存在一2．7cmX2．3crux3．1cm低回声实性团块,包膜完整,界限清楚,肿块内血流信号丰富;腹膜后、腹股沟彩超检查未见肿大淋巴结。血清肿瘤标记物pHCG、CEA和AFP未见异常。手术完整切除肿瘤,术后病检提示为睾丸神经内分泌癌。病变组织起源于神经内分泌细胞,癌细胞类圆形,胞质伊红染,核圆形,颗粒状,癌细胞呈巢状或岛状排列。癌细胞弥漫性表达突触素、嗜铬颗粒A和细胞角蛋白。结论：睾丸原发性神经内分泌癌罕见,临床表现缺乏特异性,组织学特点和免疫组化标记物是诊断该病的主要依据,需与转移性类癌和混合性类癌相鉴别。早期诊断并手术效果良好,术后应密切随访预防复发和转移。     

双侧睾丸间质细胞瘤1例报告并文献复习

目的 探讨睾丸间质细胞瘤的诊断和治疗经验.方法 报告1例双侧睾丸间质细胞瘤患者的临床资料并文献复习.结果 根据患者临床表现及相关检查,术前诊断考虑为双侧睾丸间质细胞瘤,术中快速冰冻病理检查示双侧睾丸间质细胞瘤,行睾丸肿瘤剜除术.术后随访6个月未见肿瘤复发或转移.结论 睾丸间质细胞瘤是一种较为罕见的睾丸肿瘤.术前诊断较为困难,睾酮、雌二醇等性激素水平升高及男性乳房发育等症状对睾丸间质细胞瘤的诊断有重要意义,确诊仍需依赖病理组织学检查.传统的手术方法采用根治性睾丸切除术,然而对于青春期前患者、双侧睾丸间质细胞瘤等患者,可以施行保留睾丸的肿瘤剜除术.     

Sertoli cell tumor (gonadal stromal tumor) in an infant.

An 8-mo-old boy had an orchiectomy for a Sertoli cell tumor. Malignancy was suspected histologically. He underwent a retroperitoneal lymphadenectomy and no nodes contained metastases. The histologic criteria for malignancy in Sertoli cell tumors are controversial. Individual case reports of patients with these tumors are encouraged to help establish their natural history and optimum management.     

Androblastoma and germ cell tumor of the same testis: a case report.

The rare case of a testicular androblastoma associated with a complex germ cell tumor of the same testis is reported. Gynecomastia preceded the clinical appearace of testicular swelling by 3 months, suggesting that the 2 tumors developed contemporaneously and that, in theory, the Sertoli cell tumor is probably dependent on the germ cell tumor.     

Congenital testicular juvenile granulosa cell tumor in a neonate with X/XY mosaicism

A congenital sex cord-stromal tumor of the testis with morphologic features of juvenile granulosa cell tumor is reported. The tumor occurred in an abdominal testis of a newborn infant with an X/XY karyotype and ambiguous genitalia and presented as a partially cystic mass associated with ascites. Histologically the tumor was comprised of an admixture of solid, cellular, poorly differentiated lobules mimicking graafian follicles. Residual hypoplastic testicular tissue was present at the periphery. This is the 19th reported case of testicular juvenile granulosa cell tumor and the fourth with an underlying sex chromosome anomaly, further emphasizing the relationship of this uncommon neoplasm to abnormal sexual or gonadal development.     

Sex cord tumour of the adult testis

Sex cord stromal tumors are rare and account for approximately 6% of all testicular neoplasms. We report a case of sex cord tumor composed of granulosa cells and Sertoli cells in the adult testis.