The value of CXorf67 and H3K27me3 for diagnosing germ cell tumors in central nervous system / 中华病理学杂志

Objective: To investigate immunohistochemical patterns of CXorf67 and H3K27me3 proteins in central nervous system germ cell tumors (GCTs) and to assess their values in both diagnosis and differential diagnosis. Methods: A total of 370 cases of central nervous system GCTs were collected from 2013 to 2020 at Huashan Hospital of Fudan University, Shanghai, China. The expression of CXorf67, H3K27me3 and commonly-used GCT markers including OCT4, PLAP, CD117, D2-40, and CD30 by immunohistochemistry (EnVision method) was examined in different subtypes of central nervous system GCTs. The sensitivity and specificity of each marker were compared by contingency table and area under receiver operating characteristic (ROC) curve. Results: Of the 370 cases there were 282 males and 88 females with a mean age of 19 years and a median age of 17 years (range, 2-57 years). Among the GCTs with germinoma, the proportions of male patients and the patients with GCT located in sellar region were both higher than those of GCTs without germinoma (P<0.05), respectively. CXorf67 was present in the nuclei of germinoma and normal germ cells, but not in other subtypes of GCT. H3K27me3 was negative in germinoma, but positive in the nuclei of surrounding normal cells and GCTs other than germinoma. In the 283 GCTs with germinoma components, the expression rate of CXorf67 was 90.5% (256/283), but no cases were positive for H3K27me3. There was also an inverse correlation between them (r2=-0.831, P<0.01). The expression rates of PLAP, OCT4, CD117 and D2-40 were 81.2% (231/283), 89.4% (253/283), 73.9% (209/283) and 88.3% (250/283), respectively. In 63 mixed GCTs with germinoma components, the expression rate of CXorf67 was 84.1% (53/63), while all cases were negative for H3K27me3. The expression rates of PLAP, OCT4, CD117 and D2-40 were 79.4% (50/63), 79.4% (50/63), 66.7% (42/63) and 87.3% (55/63), respectively. The 6 markers with largest area under ROC curve in ranking order were H3K27me3, CXorf67, D2-40, OCT4, PLAP and CD117 (P<0.05). Conclusions: CXorf67 and H3K27me3 have high sensitivity and high specificity in diagnosing germinoma. There is a significant inverse correlation between them. Therefore, they can both be used as new specific immunohistochemical markers for the diagnosis of GCTs.

Preoperative differentiation of benign and malignant non-epithelial ovarian tumors: clinical features and tumor markers / Diferenciação pré-operatória de tumores ovarianos não epiteliais benignos e malignos: características clínicas e marcadores tumorais

Abstract Objective To evaluate the role of clinical features and preoperativemeasurement of cancer antigen 125 (CA125), human epididymis protein(HE4), and carcinoembryonic antigen (CEA) serum levels in women with benign and malignant non-epithelial ovarian tumors. Methods One hundred and nineteen consecutive women with germ cell, sex cordstromal, and ovarian leiomyomas were included in this study. The preoperative levels of biomarkers were measured, and then surgery and histopathological analysis were performed. Information about the treatment and disease recurrence were obtained from the medical files of patients. Results Our sample included 71 women with germ cell tumors (64 benign and 7 malignant), 46 with sex cord-stromal tumors (32 benign and 14 malignant), and 2 with ovarian leiomyomas. Among benign germ cell tumors, 63 were mature teratomas, and, amongmalignant, fourwere immatureteratomas. Themost common tumors in the sex cordstromal group were fibromas (benign) and granulosa cell tumor (malignant). The biomarker serum levels were not different among benign andmalignant non-epithelial ovarian tumors. Fertility-sparing surgeries were performed in 5 (71.4%) women with malignant germ cell tumor. Eleven (78.6%) patients with malignant sex cord-stromal tumors were treated with fertility-sparing surgeries. Five women (71.4%) with germ cell tumors and only 1 (7.1%) with sex cord-stromal tumor were treated with chemotherapy. One woman with germ cell tumor recurred and died of the disease and one woman with sex cord-stromal tumor recurred. Conclusion Non-epithelial ovarian tumors were benign in the majority of cases, and the malignant caseswere diagnosed at initial stages with good prognosis. Themeasurements of CA125, HE4, and CEA serum levels were not useful in the preoperative diagnosis of these tumors.

Resumo Objetivo Avaliar o papel das características clínicas e a medida pré-operatória dos níveis séricos de CA125, HE4, e CEA em mulheres com tumores de ovário não epiteliais benignos e malignos. Métodos Cento e dezenovemulheres consecutivas comtumores ovarianos de células germinativas, do cordão sexual-estroma, e miomas ovarianos foram incluídas neste estudo. Os níveis pré-operatórios dos biomarcadores foram medidos, a cirurgia e a análise histopatológica foram realizadas. Informações sobre tratamento e recorrência da doença foram obtidas dos prontuários médicos das pacientes. Resultados Nossa amostra incluiu 71 mulheres com tumores de células germinativas (64 benignos e 7 malignos), 46 com tumores do cordão sexual-estroma (32 benignos e 14 malignos), e 2 com leiomiomas ovarianos. Entre os tumores benignos de células germinativas, 63 eram teratomas maduros, e, entre os malignos, quatro eram teratomas imaturos. Os tumores mais comuns do grupo do cordão sexual-estroma foram fibromas (benignos) e tumores de células da granulosa (malignos). Os níveis séricos dos biomarcadores não diferiram entre os tumores de ovário não epiteliais benignos e malignos. A cirurgia preservadora de fertilidade foi realizada em 5 (71,4%) mulheres com tumores malignos de células germinativas. Onze (78,6%) mulheres com tumores do cordão sexual-estromamalignos foram tratadas comcirurgia preservadora de fertilidade. Cinco (71,4%)mulheres com células germinativas e apenas 1 (7,1%) com tumor do cordão sexual-estroma foram tratadas com quimioterapia. Uma mulher com tumor de células germinativas recidivou e morreu da doença. Uma mulher com tumor do cordão sexual-estroma recidivou. Conclusão Os tumores de ovário não epiteliais foram benignos namaioria dos casos e os malignos foram diagnosticados em estágios iniciais, com bom prognóstico. A medida dos níveis séricos de CA125, HE4, e CEA não foram úteis no diagnóstico préoperatório desses tumores.

The relationship of neutrophil to lymphocyte ratio with testicular cancer

ABSTRACT Purpose: To assess the relationship between testicular germ cell tumors (TGCT) and neutrophil to lymphocyte ratio (NLR) and to determine whether this ratio can be used as a serum tumor marker. Material and Methods: Sixty-one patients with testicular germ cell tumors were included into the study. Patients were grouped as localized and non-localized. Histologically patients were categorized as seminoma and nonseminomatous germ cell tumors. Complete blood cell count was measured the day before surgery and at the postoperative 1st month. Preoperative and postoperative mean NLR values were compared. Results: Thirty-six patients (59%) had seminomas and 25 patients (41%) had nonseminomatous testicular cancer. Forty-five patients (73.8%) had localized and 16 patients (26.2%) had non-localized testicular cancer. There was a statistically significant difference between preoperative and postoperative mean NLR of the localized patients (p=0.001) but no such difference was detected for non-localized patients (p=0.576). Nineteen patients with localized seminomas had normal preoperative serum tumor markers. There was a significant difference between preoperative and postoperative mean NLR in this group of patients (p=0.010). Twenty-six patients with localized tumors had preoperative increased serum tumor markers which normalized after orchiectomy. Mean NLR of these patients significantly decreased from 3.10±2.13 to 1.62±0.59 postoperatively (p=0.010). Conclusions: NLR appears to be a useful marker for TGCT. It is successful in predicting localized and non-localized disease in early postoperative period.

Tumores ováricos en niñas y adolescentes / Ovarían masses in infant-juvenile age

Introducción. El tratamiento quirúrgico adecuado para las pacientes pediátricas con lesiones ováricas es heterogéneo, y en las niñas es conveniente conservar los ovarios. El objetivo de este estudio es analizar los hallazgos relacionados con un grupo de pacientes a las que se operó por lesiones ováricas. Pacientes y métodos. Un estudio retrospectivo realizado durante 13 años con 56 pacientes menores de 17 años. Estas pacientes se dividieron en 3 grupos según el diagnóstico de patología ovárica: 25 tenían lesiones funcionales (quistes y torsión), 18 tenían lesiones ováricas epiteliales y 13 tenían tumores germinales. Se comparó a estos tres grupos en términos de menarquia, torsión, edad, duración, tamaño, dolor, tumor, vómitos, menstruación irregular, ubicación y tipo de operación. Resultados. Enestosgrupos,fueronmásfrecuentes los quistes foliculares, los cistoadenomas serosos y los teratomas. La media de edad de las pacientes fue de 12,18 ± 4,84 años. Los síntomas y signos más frecuentes fueron dolor (85,7%) e hinchazón (37,5%) en la región abdominopélvica. Se observó torsión en 21 pacientes (37,5%); la media del tamaño del tumor fue de 10,46 ± 6,55 cm. Se realizó una salpingoovariectomía (SO, por sus siglas en inglés) en 38 pacientes y una resección del quiste (CE, por sus siglas en inglés) en 18 pacientes. Se observaron más casos de torsión en las pacientes premenárquicas que en las menárquicas y en el grupo de lesiones funcionales. Se realizaron más resecciones quirúrgicas en el grupo de lesiones funcionales, y más SO en los grupos de lesiones epiteliales y tumores germinales. Conclusión. Se considera que la torsión y las patologías ováricas funcionales son frecuentes en la edad premenstrual, y las lesiones malignas son muy raras en todos los grupos etarios, por lo que se recomienda preservar la fertilidad en la cirugía.

Introduction. The appropraite surgical treatment to pediatric patients with ovarian lesions are heterogeneous and ovarian preservation is desirable in children. The aim of this study is to the discuss findings related to a set of patients who were operated on for ovarian lesions. Patients and methods. A retrospective study carried out in 13 years on 56 patients under the age of 17. These patients were divided into 3 groups according to ovarian pathologic diagnosis: 25 with functional (cyts and torsion), 18 with epithelial ovarian lesions and 13 with germ cell tumours. These three groups were compared in terms of menarche, torsion, age, duration, size, pain, mass, vomiting, irregular menstruation, location and operation type. Results. Follicle cysts, serous cyst adenomas and teratomas were the most common in these groups. The mean age of the patients was 12.18+4.84 years. The most common symptoms and signs were abdominal-pelvic pain (85.7%) and swelling(37.5%). Torsion was seen in 21 patients (37.5%), mean mass size was found to be 10.46+6.55 cm. A salpingo-oophorectomy (SO) was performed in 38 patients and cyst excision (CE) was performed in 18 patients. In premenarcheal cases, torsion was seen more in menarcheal cases and in the functional lesion group. CE was performed more often in the functional and t SO was performed often in the epithelial and germ cells groups. Conclusion. Torsion and functional ovarian pathologies are thought to be common in premenstrual ages and malign lesions are very rare in all age groups so we recommend ovarian protective surgery should be preferred.

Yolk Sac Tumor of the Temporal Lobe: Case Report

Germ cell tumors of the central nervous system (CNS) are usually located along the midline. Yolk sac tumor is a rare germ cell tumor very uncommonly located outside the midline, and, in such cases, it can be mistaken with other primary tumors. We report a case of a 32-year-old male patient who presented with a right temporal lobe tumor suggestive of a high grade glioma. He was submitted to a right temporal lobectomy with complete tumor removal. The histological exam revealed a germ cell tumor (later confirmed to be a yolk sac tumor). The search for a primary tumor outside of the CNS (including a positron emission tomography scan) was negative, making this a primary temporal lobe yolk sac tumor. The patient was submitted to chemotherapy and radiotherapy, but died 7 months after the surgery.

Yolk sac primary tumor of mediastino: a rare case in a young adult / Tumor de saco vitelínico primário do mediastino: raro caso em um adulto jovem

ABSTRACT Germ cell tumors are rare neoplasms that mostly occur in the gonads, although they can also affect other body sites, especially the anterior mediastinum (50 to 70% of all extragonadal germ cell tumors). We report a case of a primary mediastinal yolk sac tumor, a rare and aggressive germ cell tumors subtype. This was a 38-year-old man who was admitted to Hospital do Servidor Público Estadual "Francisco Morato de Oliveira", complaining about dyspnea and dry cough for 1 year. The computed tomography scan of his chest revealed a large mass in the anterior mediastinum with heterogeneous enhancement to the contrast associated with pleural effusion. There were also high serum levels of alpha-fetoprotein. After neoadjuvant chemotherapy, the patient underwent surgical resection of the mass, followed by pathological examination, which confirmed a primary mediastinal yolk sac tumor, a nonseminomatous subtype of germ cell tumors. Primary mediastinal yolk sac tumors have poor prognosis, despite advances in therapy with surgical resection and cisplatin-based chemotherapy. This poor prognosis is due to the degree of invasion and unresectability in most patients by the time of the diagnosis.

RESUMO Os tumores de células germinativas são neoplasias raras que acometem mais frequentemente as gônadas, embora possam também ocorrer em outras localizações do corpo, destacando-se o mediastino anterior (50 a 70% de todos os tumores de células germinativas extragonadais). No presente artigo, relatamos um caso de tumor de saco vitelínico mediastinal primário, de subtipo raro e agressivo de tumor de células germinativas. Tratava-se de um homem, 38 anos, admitido no Hospital do Servidor Público Estadual "Francisco Morato de Oliveira", com quadro de dispneia e tosse seca há 1 ano. Na investigação clínica, foi solicitada tomografia computadorizada de tórax, que mostrou volumosa massa no mediastino anterior com realce heterogêneo ao meio de contraste associada a derrame pleural. Havia ainda aumento dos níveis séricos da alfafetoproteína. Após quimioterapia neoadjuvante pré-operatória, o paciente foi submetido à ressecção cirúrgica, seguida de estudo anatomopatológico da peça, no qual demonstrou tratar-se de um tumor de saco vitelínico primário do mediastino. Os tumores de saco vitelínicos primários do mediastino têm prognóstico reservado, apesar do avanço na terapêutica com a ressecção cirúrgica e a quimioterapia à base de cisplatina. Isto se deve ao grau de invasão e de irressecabilidade na maioria dos pacientes no momento do diagnóstico.

White blood cell counts and neutrophil to lymphocyte ratio in the diagnosis of testicular cancer: a simple secondary serum tumor marker

ABSTRACT Purpose The aim of the study was to investigate white blood cell counts and neutrophil to lymphocyte ratio (NLR) as markers of systemic inflammation in the diagnosis of localized testicular cancer as a malignancy with initially low volume. Materials and Methods Thirty-six patients with localized testicular cancer with a mean age of 34.22±14.89 years and 36 healthy controls with a mean age of 26.67±2.89 years were enrolled in the study. White blood cell counts and NLR were calculated from complete blood cell counts. Results White blood cell counts and NLR were statistically significantly higher in patients with testicular cancer compared with the control group (p<0.0001 for all). Conclusions Both white blood cell counts and NLR can be used as a simple test in the diagnosis of testicular cancer besides the well-known accurate serum tumor markers as AFP (alpha fetoprotein), hCG (human chorionic gonadotropin) and LDH (lactate dehydrogenase).

Tailored therapy and long-term surveillance of malignant germ cell tumors in the female genital system: 10-year experience / 부인종양

OBJECTIVE: To explore the appropriate treatment of malignant germ cell tumor (MGCT) in the female genital system, and to analyze the factors influencing both therapeutic response and survival outcome. METHODS: A cohort of 230-Chinese women diagnosed with MGCT of the genital system was retrospectively reviewed and prospectively followed. The demographic and pathological features, extent of disease and surgery, treatment efficiency, recurrence and survival were analyzed. RESULTS: MGCTs from different genital origins shared a similar therapeutic strategy and response, except that all eight vaginal cases were infantile yolk sac tumors. The patients' cure rate following the initial treatment, 5-year overall survival and disease-free survival (DFS) were 85.02%, 95.00%, and 86.00%, respectively. Although more extensive excision could enhance the remission rate; it did not improve the patients' survival. Instead, the level of the medical institution, extent of surgery and disease were independent prognostic factors for relapse (p<0.05). Approximately 20% of patients had recurrent or refractory disease, more than half of whom were in remission following secondary cytoreductive surgery with salvage chemotherapy. CONCLUSION: Fertility-sparing surgery with or without standardized PEB/PVB (cisplatin, etoposide/vincristine, and bleomycin) chemotherapy is applicable for female MGCTs of different origins. Comprehensive staging is not required; nor is excessive debulking suggested. Appropriate cytoreduction by surgery and antineoplastic medicine at an experienced medical institution can bring about an excellent prognosis for these patients.

The incidence and histological characteristics of intratubular germ cell neoplasia in postpubertal cryptorchid testis

PURPOSE: It is well known that testicular germ cell tumors arise with increased frequency in patients with cryptorchidism. In addition, intratubular germ cell neoplasia (ITGCN) is a precursor lesion to testicular germ cell tumor. Approximately 50% of patients with ITGCN will develop an invasive of testicular germ cell tumors within 5 years. Therefore, we evaluated that the incidence of ITGCN in postpubertal cryptorchidism. MATERIALS AND METHODS: Between January 2002 and August 2012, orchiectomy specimens from 31 postpubertalpatients (aged 12 or over) with cryptorchid testis were reviewed. The specimens were evaluated for ITGCN using immunohistochemical stains of placental-like alkaline phosphatase and Oct 3/4 with routine hematoxylin-eosin stain. Additionally, the degree of spermatogenesis was assessed using the Johnsen score. RESULTS: Mean age was 34 years (range, 17 to 74 years) at surgery. All patients were diagnosed as unilateral cryptorchidism. One patient (3.2%) of 20-year-old had ITGCN in surgical specimen with all positive markers. Histological assessment of spermatogenesis showed that mean Johnsen score was 3.42 (range, 1 to 9). Majority of patients (27 of 31) presented impaired spermatogenesis with low Johnsen score lesser than 5. CONCLUSIONS: Considering the risk of malignancy and low spermatogenesis, we should perform immunohistochemical stains and discuss preventative orchiectomy for the postpubertal cryptorchidism.

CT Findings of Intrarenal Yolk Sac Tumor with Tumor Thrombus Extending into the Inferior Vena Cava: A Case Report

Yolk sac tumor (YST) is a rare germ cell neoplasm of childhood that usually arises from the testis or ovary. The rare cases of YST in various extragonadal locations have been reported, but the primary intrarenal YST is even more uncommon. Here, we report a case of a primary intrarenal YST with tumor thrombus of the inferior vena cava and left renal vein in a 2-year-old boy, with an emphasis on the CT features. To our knowledge, this is the first reported case of an intrarenal YST with intravascular involvement.

Struma ovarii maligno / Malignant Struma ovarii

O "Struma ovarii" é um tumor ginecológico raro, sendo de malignização e metástases incomuns. Constituído predominantemente por tecido tireoideano, apresenta maior incidência aos 50 anos. Apresentamos o caso de uma paciente de 41 anos com história prévia de teratoma tratado, que desenvolve dor pélvica e tumoração abdominal. Operada, concluiu-se tratar de "Struma ovarii" maligno com metástases. Após a radioablação com I-131, encontra-se sob controle clínico. Apesar de sua raridade, o "Struma ovarii" possui bom prognóstico, devendo ser lembrado no diagnóstico diferencial dos tumores ovarianos.

Development of Acute Megakaryoblastic Leukemia with Isochromosome (12p) after a Primary Mediastinal Germ Cell Tumor in Korea

The association of hematological malignancies with a mediastinal germ cell tumor (GCT) is very rare. We report one case of a young adult male with primary mediastinal GCT who subsequently developed acute megakaryoblastic leukemia involving isochromosome (12p). A 25-yr-old man had been diagnosed with a mediastinal GCT and underwent surgical resection and adjuvant chemotherapy. At 1 week after the last cycle of chemotherapy, his peripheral blood showed leukocytosis with blasts. A bone marrow study confirmed the acute megakaryoblastic leukemia. A cytogenetic study revealed a complex karyotype with i(12p). Although additional chemotherapy was administered, the patient could not attain remission and died of septic shock. This case was definitely distinct from therapy-related secondary leukemia in terms of clinical, morphologic, and cytogenetic features. To our knowledge, this is the first case report of a patient with mediastinal GCT subsequently developing acute megakaryoblastic leukemia involving i(12p) in Korea.

Ovarian Cancer during Pregnancy: Clinical and Pregnancy Outcome

The aim of this study is to evaluate the clinical feature and pregnancy outcome in patients with ovarian cancer diagnosed during pregnancy. We retrospectively analyzed the medical records of 27 patients diagnosed with ovarian cancer during pregnancy at Cheil General Hospital & Women's Healthcare Center from January 1996 to December 2006. Mean age of the patients was 29.1 yr (range 23-40), and a mean follow-up period was 57 months (range 7-112 months). Of 27 patients, 15 (55.5%) had borderline malignancies, 7 (25.9%) had epithelial malignancies and 5 (18.6%) had germ cell tumors. A total of 26 patients received a conservative surgery preserving pregnancy. The mean time for surgical intervention during pregnancy was 20 weeks of gestational age. Of the 27 patients, 26 had full term delivery of a healthy baby without any congenital malformation. Only one patient with epithelial ovarian cancer had a relapse at 19 months after the first conservative operation with adjuvant chemotherapy. There were few data for managing patients with ovarian cancer diagnosed during pregnancy. This study results could help establish a guideline for management of ovarian malignancy complicating pregnancy.

[Malignant ovarian mixed germ cell tumor: a rare case]

Mixed germ cell tumors of the ovary, uncommon but aggressive tumors are seen most often in young women or adolescent girls, and are generally curable if found and treated early. Use of chemotherapy after initial surgery has dramatically improved the prognosis for many females with these tumors. This study reports a case of successful pregnancy in a patient with malignant ovarian mixed germ cell tumor. The patient was an 18-year old nulliparous woman who had pelvic pain, menstruation delay, right Adnex mass and positive B-HCG. She was found to have a malignant mixed germ cell tumor comprising of both embryonal carcinoma and immature teratoma. Treatment consisted of salpingo-oophorectomy with adjunctive chemotherapy. In view of malignant germ cell tumors occurring almost exclusively in young females, preservation of their ovarian function and fertility was an important issue. The present study, confirmed that normal gonadal function and fertility are possible after conservative surgery for ovarian germ cell malignancies, even with adjuvant chemotherapy

Suprasellar germ cell tumor in a dog

A case of suprasellar germ cell tumor is described in a 6-year -old Airedale Terrier bitch that presented behavioral changes and abrupt onset of blindness. The neoplasm compressed the ventral surface of the cerebrum from the level of basal ganglia to the mesencephalon. Histologically the neoplasm consisted of nests and trabeculae of round to polygonal cells that occasionally surround tubules and cysts formed by columnar cells. Neoplastic cells are immunopositive for cytokeratin and alpha-fetoprotein. The diagnosis was based on location, histological appearance and on the immunohistochemical staining.

É descrito um caso de tumor de células germinativas supra-selar em numa cadela Airedale Terrier de seis anos de idade, que apresentou transtornos do comportamento e aparecimento abrupto de cegueira. O neoplasma comprimia a superfície ventral do cérebro desde a altura dos núcleos basais até o mesencéfalo. Histologicamente, o neoplasma consistia de ninhos e trabéculas de células redondas ou poligonais que ocasionalmente arranjavam-se ao redor de túbulos e cistos formados por células colunares. As células neoplásicas foram positivas na imunoistoquímica para citoqueratina e a-fetoproteína. O diagnóstico foi feito com base na localização do tumor, no seu aspecto histológico e nos resultados da marcação imunoistoquímica.

Mixed germ cell tumours of the pineal region

Intracranial mixed germ cell tumours, particularly with the occurrence of germinoma and teratoma components, are very rare. On many occasions, the diagnosis is only reached after a second surgical resection of what is believed to be a tumour recurrence. The authors report two young adolescent males who presented with headache, vomiting and Parinaud's syndrome. Cranial CT and MRI scans demonstrated a large heterogeneous pineal region tumour. Through a supracerebellar infratentorial approach, in both patients, a complete excision of the tumour was achieved of a histopathologically proven mixed germ cell tumour, predominantly teratoma with elements of germinoma. The patients received adjuvant chemotherapy and radiotherapy and remained tumour free during their follow-up. The authors discuss the management of this rare entity and emphasize the importance of detailed histopathological examination

Embryonal sarcoma of liver: an undifferentiated rare primary solid and mucoid to cystic tumour.

Undifferentiated (embryonal) sarcoma of the liver is a rare primary malignant tumor of the liver occurring almost exclusively in childhood with characterisitic findings on gross and microscopy. Careful sampling of the tumor shows various immature undifferentiated elements and infiltration of the tumor into the surrounding capsule. CT findings could be suggestive of a hamartoma, as in our case. We report a case of hepatic embryonal sarcoma in a ten-year old male child presenting with an abdominal mass.