Ollier's disease and juvenile ovarian granulosa tumor

The authors report one case of Ollier's disease in a 12 year-old girl in whom the occurrence of an ascites revealed a secretant juvenile granulosa cell tumor some years later. The tumor was right-sided, homolateral to the hemicorporal side involved by enchondromatosis. Five other cases of this association were previously reported. Attention is drawn not only to the risk of chondrosarcomatous change but also to the possible recurrence of ovarian tumor in the year following ovariectomy.     

Ovarian granulosa cell tumors in childhood

Granulosa cell tumors (GCT) of the ovary are prepubertal in 5% of the patients. In girls less than 20 years old, 80% of GCTs differ from those among adults. These juvenile granulosa cell tumors (JGCTs) are usually benign. GCTs belong to ovarian sex cord-stromal tumors, the more common ovarian tumors being epidermal and germinal. The etiology of GCT remains unknown. Most young children with GCT present with precocious pseudopuberty. Among adolescents GCT often causes menstrual irregularities, virilization, abdominal swelling, and pain. When JGCT is limited to the ovaries the outcome is excellent with only salpingo-oophorectomy. However, more widely spread tumors are difficult to treat and cause mortality. Cisplatin-containing chemotherapy can induce remissions in adult GCTs. Estrogens and peptide hormones, i.e., inhibin, are useful in the follow-up of the patients. The authors describe 3 children with GCT and review current data on this rare tumor from molecular biology to clinical aspects.     

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.     

Ovarian granulosa cell tumors: an unusual cause of precocious thelarche

Precocious thelarche usually results from a physiological process but can sometimes be the first sign of precocious pseudopuberty. Ovarian granulosa cell tumors are highly unusual in childhood, appearing as precocious puberty in most prepuberal patients. During adolescence these tumors may cause menstrual irregularities, virilization and abdominal pain. Their malignancy is low and surgical treatment is usually curative if the tumors are limited to the ovaries. More advanced stages require chemotherapy, are difficult to cure and produce high mortality. We present the case of a 16-month-old girl with a granulosa cell tumor who presented with progressive precocious thelarche over 1 month that was satisfactorily resolved after resective surgery. This case demonstrates that other causes of puberal development should be investigated when precocious thelarche with fast progression is observed, with special attention paid to tumoral disease in the differential diagnosis.     

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.     

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.     

Childhood ovarian juvenile granulosa cell tumor: a retrospective study with 3 cases including clinical features, pathologic results, and therapies

Ovarian juvenile granulosa cell tumor (JGCT) is an extremely rare sex cord-stromal tumor that is most commonly encountered in prepubertal girls and young women. In this study, 3 cases of childhood JGCT are reported. The main causes of hospitalization were abnormal abdominal signs and syndromes. Imaging findings indicated masses with multiple cysts and solid components in the abdominal cavities. Diagnosis was confirmed by histopathology. Tumors were excised completely, and all cases have a good outcome in the follow-up (2 to 13 y) without any adjuvant chemotherapy or radiotherapy. This data suggests that children with JGCT might have a variety of initial clinical manifestations. Pathologic examination especially immunohistochemical staining could help to diagnose JGCT. For children, adjuvant treatment may not be necessary if the tumor can be excised completely in early stage.     

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.     

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.     

Pseudo-precocious puberty caused by a juvenile granulosa cell tumour secreting androstenedione, inhibin and insulin-like growth factor-I

We report a female child who presented at age 3.92 years with a 2-year history of consonant pubertal development caused by a large right-sided ovarian juvenile granulosa cell tumour (JGCT). Although JGCTs causing pseudo-precocious puberty have been previously described, they remain rare and endocrine data are often incomplete. In this case the tumour was associated with raised serum oestradiol, androstenedione, inhibin and IGF-I. Histological changes were consistent with JGCT. Immunohistochemical studies revealed positive reactivity to MIC-2, inhibin, melan A, IGF-I and IGFBP-2.     

The chemotherapy of granulosa cell tumors of the ovary: Experience of the wisconsin clinical cancer center

We reviewed the clinical records of 32 patients with ganulosa cell tumor of the ovary treated at the Wisconsin Clinical Cancer Center (WCCC) between 1970 and 1982. Eleven of these patients were treated with one or more chemotherapeutic regimens, yielding a total of 22 treatment trials. Objective response was observed in 7 of 17 evaluable treatment trials (41%). The response to chemotherapy could not be assessed in five treatment trials due to the concomitant administration of radiotherapy. We conclude that granulosa cell tumor of the ovary is responsive to chemotherapy. However, the optimal chemotherapeutic regimen for this rare neoplasm remains to be established on the basis of prospective clinical trials.     

Therapy of juvenile rheumatoid arthritis]

The correct drug treatment of JRA must consider the course and the subtype of the disease. Nonsteroidal antiinflammatory drugs are the first choice treatment, especially the recent ones which are more active and less toxic. The slow-acting antirheumatic drugs are the second choice treatment and must be employed in the chronically active stages of the disease; good results have been obtained with sulphasalazine and methotrexate both on clinical features and on blood biochemistry with relatively scarce side effects. Thymic hormones, cyclosporin A and intravenous immunoglobulins, though not yet widely experienced, can represent a worthwhile alternative to standard treatment in carefully selected cases. Steroids must be used only in special cases (particularly aggressive systemic JRA, carditis, severe anemia and those patients who fail to respond to usual treatments) and must be withdrawn as soon as possible to avoid adverse effects and steroid-addiction. Intraarticular long-acting steroids are the first choice treatment for rheumatoid monoarthritis.