Juvenile granulosa cell tumor of an intraabdominal testis

Juvenile granulosa cell tumor of the testis is a distinct form of sex cord-stromal tumor of neonates and infants [1]. This tumor comprises a significant percentage of testicular tumors in baby boys. We present a patient who had preoperative imaging studies.     

Juvenile granulosa cell tumor

A 2-year-old girl presented with isosexual precocity, and a juvenile granulosa cell tumor of the left ovary was removed. The tumor was unusual in that striated muscle differentiation of the tumor cells was demonstrated. This suggests that the mesenchymal cells retain the potential for divergent differentiation.     

Prenatal diagnosis of juvenile granulosa cell tumor of the testis

Juvenile granulosa cell tumor is a rare benign neoplasm of the testicular stroma that accounts for 1-5% of all prepubertal testis tumors [Metcalfe PD, Farivar-Mohseni H, Farhat W, McLorie G, Khoury A, Bagli DJ. Pediatric testicular tumors: contemporary incidence and efficacy of testicular preserving surgery. J Urol 2003;170:2412-2416; Ross JH, Rybicki L, Kay R. Clinical behavior and a contemporary management algorithm for prepubertal testis tumors: a summary of the prepubertal testis tumor registry. J Urol 2002;168:1675-1679]. A prior case series retrospectively identified a cystic testis tumor on prenatal ultrasound images which was subsequently diagnosed as a juvenile granulosa cell tumor [Bryan DE, Cain MP, Casale AJ. Juvenile granulosa-theca cell (sex cord-stromal) tumor of the infant testis. J Urol 2003;169:1497-1498]. We report a case of a prenatally diagnosed testis tumor which was subsequently diagnosed as a juvenile granulosa cell tumor.     

Juvenile granulosa cell tumor of the infant testis: case report with ultrastructural observations

This report describes the light-microscopic and ultrastructural features of a juvenile granulosa cell tumor of infant testis. Microscopic examination revealed a macrofollicular patterns simulating the preovulatory Graafian follicle and the juvenile granulosa cell tumor of the ovary. Ultrastructure confirmed three cell types: granulosa, theca interna, and externa, with occasional luteinized cells lacking crystalloids of Reinke. Charcot-Bottcher crystalloids were not detected, though rare cells contained a complex arrangement of filaments. An ultrastructural comparison was carried out with infant testes (2 cases), preovulatory Graafian follicle (1 case), juvenile granulosa cell tumor of ovary, adult granulosa cell tumor of ovary, and adult Sertoli cell tumor of testis and ovary. Ultrastructural similarities were noted between the present case and primitive Sertoli cells, preovulatory granulosa cells, and juvenile granulosa cell tumor of ovary. This may reflect the common histogenesis of Sertoli/granulosa cells from the common specialized gonadal stroma.     

MRI findings of bilateral juvenile granulosa cell tumor of the testis in a newborn presenting as intraabdominal masses

Juvenile granulosa cell tumor (JGCT) of the testis is a rare benign tumor that typically presents as a relatively small (<2 cm) unilateral scrotal mass in neonates or infants. Bilateral JGCT of the testes presenting as large intraabdominal masses in the neonate is very rare. Utilizing preoperative MRI findings, we report a rare case of bilateral JGCT of the testes presenting as large multiseptated abdominal masses originating from undescended intraabdominal testes in a neonate.     

Galactorrhea-associated granulosa cell tumor in a child

Granulosa cell tumor of the ovary is a rare form of ovarian cancer in children. An 11-year-old girl was admitted with complaints of galactorrhea and abdominal mass. Abdomino-pelvic ultrasound and computed tomography revealed an ovarian tumor. Her prolactine and estradiol levels were increased but luteinizing hormone and follicle-stimulating hormone were decreased. An exploratory laparotomy revealed a giant solid mass, which was completely removed and determined as juvenile granulosa cell tumor. The clinical, hormonal, and radiological findings and the therapy of galactorrhea associated with granulosa cell tumor in a child are discussed. To our knowledge, this is first time it has been described in childhood.     

Sertoli cell tumor of the testis in children: reevaluation of a rarely encountered tumor

Testis tumors are uncommon in childhood, and they differ from adult tumors in terms of histology and frequency. Sertoli cell tumors appear in children before 1 year of age. They are more frequently benign, but because of the absence of specific signs of malignancy, treatment consists of radical orchiectomy, sometimes followed by radiotherapy or chemotherapy based on histologic analysis. Malignancy is confirmed by the presence of metastasis or many mitosis and anaplastic cells. Children must be followed for several years to be sure of the absence of relapse or progression. In the authors' hospital, of 13 testis tumors diagnosed since 1996, only 2 were Sertoli cell tumors. It would be helpful to have an algorithm for the management of testis tumors, outlining how to make the diagnosis of malignancy and which treatment and follow-up to pursue.     

Successful multimodality therapy of recurrent multifocal juvenile granulosa cell tumor of the ovary

Juvenile granulosa cell tumor (JGCT) of the ovary, a rare pediatric cancer, carries a very poor prognosis in advanced and recurrent cases. A 10-year-old girl with stage IA JGCT, initially treated with resection only, presented with extensive unresectable multifocal pelvic recurrence. She underwent surgery, chemotherapy (cisplatin/paclitaxel alternating with cisplatin/cyclophosphamide/etoposide/bleomycin), myeloablative chemotherapy (carboplatin/etoposide/melphalan) with autologous bone marrow transplant, and pelvic radiation. She tolerated therapy well and is in complete remission 69 months after her recurrence.     

Juvenile dermatomyositis: diagnosis and treatment]

Juvenile dermatomyositis is a rare inflammatory myopathy with characteristic skin manifestations and muscular weakness. Muscle biopsy is important for establishing the diagnosis. Four patients with juvenile dermatomyositis were studied retrospectively. Steroids remain the first line treatment. Corticosteroids resistance is the primary indication for the use of intravenous immunoglobulins or immunosuppressive drugs. Further studies are necessarily aimed at finding biological markers to select and guide new therapeutical approaches for those patients.     

腹腔出血的幼儿卵巢颗粒细胞瘤一例

患儿女,2岁2个月,因腹痛伴发热2d入院.检查:T:37.5℃、P:98次/min、R:25次/min、BP:100/67 mm Hg,贫血貌,乳房幼儿型、无明显发育.腹部膨隆、压痛、反跳痛、轻度肌紧张,可触及一直经约14 cm的类球状肿物,质地中等,移动度尚可,有触痛.外院腹部超声:腹腔11 cm×13 cm×14 cm不均质回声,伴腹腔积液,最深约5.9 cm.     

Cystic adult granulosa cell tumor causing precocious pseudopuberty in a six-year-old girl

A very rare occurrence of adult granulosa cell tumor of the ovary (not of the juvenile type) causing precocious pseudopuberty in a six-year-old girl is described. An additional feature of interest was that the tumor appeared entirely cystic. To our knowledge, this condition in such a young premenarchal patient has not been reported previously.