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[Study of twenty-three cases of pediatric testicular tumors].

Twenty-three cases of testicular tumor (10; malignant, 13; benign) in childhood are reported. The age at diagnosis was between 3 months old and 8 years old. Twelve patients visited the clinic within a month of first appearance of symptoms. All but one patient received high orchiectomy. One patient had resection of tumor because of its benign property. Eight of the 10 patients with malignancies received 30-50Gy of retroperitoneal radiation, including one patient (stage IIa) with retroperitoneal lymph node dissection. Two patients (stage I and IIa) with post-operative metastasis in lungs, brain and mediastinal lymph nodes were treated with chemotherapy repeatedly, but they died. Our latest therapy is; 1) high orchiectomy recommended for benign and stage I malignant testicular tumors in childhood, 2) retroperitoneal radiation and/or lymph node dissection limited for patients with stage II malignancy, and 3) Systemic chemotherapy for stage III malignant tumors. At least three years of follow up would be needed. Those who received radiation or chemotherapy should be followed longer.

Age of Onset↗

Detection of chromosome aberrations in paraffin sections of seven gonadal yolk sac tumors of childhood.

Yolk sac tumors are the most frequent kind of malignant pediatric germ cell tumor and may have a fundamentally different pathogenesis than adult germ cell tumors. Since few cytogenetic studies have been performed so far, in situ hybridization was applied to interphase cell nuclei of seven gonadal yolk sac tumors of childhood in routine paraffin-embedded tissue sections. The panel of chromosome-specific DNA probes was selected on the basis of their relevance in adult germ cell tumors and consisted of five DNA probes specific for the (peri)centromeric regions of chromosomes 1, 8, 12, 17 and/or X and/or one DNA probe specific for the subtelomeric region of chromosome 1 (p36.3). As in adult germ cell tumors, all pediatric gonadal yolk sac tumors had an increased incidence of numerical chromosome aberrations. All tumors showed an overrepresentation of at least three chromosomes. Gains of chromosome 12, which is highly specific in adult germ cell tumors, were diagnosed in six pediatric gonadal yolk sac tumors. The DNA indices determined in the paraffin-embedded tumor material correlated well with the in situ hybridization findings. A chromosome was either over- or underrepresented, compared with the corresponding DNA indices, in only a few cases. The short arm of chromosome 1 in adult germ cell tumors is often involved in structural aberrations. In pediatric germ cell tumors, the short arm of chromosome 1 is also a nonrandom site of structural aberrations. Moreover, the presence of a deletion at 1p36.3 in four out of five tumors suggests that the loss of gene(s) in this region is an important event in the pathogenesis of gonadal yolk sac tumors of childhood.

Adult↗

Somatic isoform of angiotensin I-converting enzyme in the pathology of testicular germ cell tumors.

Retained fetal expression of angiotensin I-converting enzyme (ACE, CD143) has recently been shown in intratubular germ cell neoplasms (IGCN) and invasive germ cell tumors (GCT), suggesting the somatic isoform (sACE) as a characteristic component of neoplastic germ cells. We analyzed the distribution of sACE in 159 testicular GCT, including 87 IGCN. sACE protein was determined by immunohistochemistry (MAb CG2) on routinely formalin-fixed and paraffin-embedded tissue sections, supplemented by mRNA expression analysis using in situ hybridization. These data were compared with those obtained by germ cell/placental alkaline phosphatases (PIAP; MAbs PL8-F6 and 8A9) employing an uniform score system for the evaluation of immunoreactivity (IRS; possible values from 0 to 12). Expression of sACE and PIAP was found in all 87 analyzed IGCN (IRS > 4, median IRS of 12). Heterogeneous staining patterns were not related to the type of adjacent GCT but correlated with low expression in adjacent seminomas (P =.032 for sACE; P =.005 for PIAP). Both sACE and PIAP often showed a decreased and more heterogeneous but still moderate expression in 91 classic seminomas (median IRS of 8) and were completely absent in tumor cells of spermatocytic seminomas. Despite all similarities, we found sACE and PIAP differently regulated during GCT progression. This was documented by a well-preserved expression of either sACE or PIAP or both in all classic seminomas, low PIAP immunoreactivity in metastasis of seminomas, and completely diverging expression patterns in nonseminomatous GCT. Our findings underline the close molecular relationship between IGCN and seminoma, and suggest sACE as an appropriate marker for seminomatous differentiated tumors. HUM PATHOL 31:1466-1476.

Adolescent↗

Occult malignancy in neonatal sacrococcygeal teratomas. A report from a Combined Pediatric Oncology Group and Children's Cancer Group study.

PURPOSE: Six children who had mature or immature sacrococcygeal teratomas diagnosed in the newborn period have since been registered on a Pediatric Oncology Group/Children's Cancer Group germ cell study with recurrent malignant neoplasia [pure yolk sac tumor (YST) or teratoma with yolk sac elements]. RESULTS: Four of the children have responded to therapy, one has died, and one has been lost to follow-up. Review of the slides from five of the original tumors identified microscopic foci of YST in four. CONCLUSIONS: Detection of such foci in neonatal tumors is important because serum alpha-fetoprotein concentrations may not be helpful since they may normally be high in the newborn period due to fetal production.

Antineoplastic Combined Chemotherapy Protocols↗

Sacrococcygeal tumors in infancy and childhood; a retrospective histopathological review of 85 cases.

This study retrospectively examines the spectrum of sacrococcygeal tumors reported in a tertiary paediatric pathology department during a 15-year period. There were 85 sacrococcygeal tumors identified in total, including 79 (93%) sacrococcygeal germ cell tumors, of which 62 (78%) were benign, whereas 17 (22%) contained malignant yolk sac tumor elements. The median age at examination in cases with malignant elements present was significantly greater than in those with benign sacrococcygeal teratoma only (median 2 years, range birth--3 years versus median 1 week, range birth--10 years, respectively; p < .01). Of the 85 cases of total sacrococcygeal lesions 6 (7%) represented pathologies other than sacrococcygeal teratoma, including one case each of neuroblastoma, ganglioneuroma, myxopapillary ependymoma, primitive neuroectodermal tumor, lipomatous tumor, and unclassifiable inflammatory tumor. Of these 6 cases 3 were malignant (50%) compared with 17 of the 79 cases of sacrococcygeal germ cell tumors (22%; Z =1.59, p = .08). The median age in the group of non-germ-cell sacrococcygeal masses was 3 years (range 5 months to 13 years).

Adolescent↗

Primary mediastinal yolk sac tumor in a 66-year-old woman.

OBJECTIVE: To report a rare case of nonseminomatous extragonadal germ cell tumor in a 66-year-old female with primary yolk sac tumor of the mediastinum. CLINICAL PRESENTATION AND INTERVENTION: A 66-year-old female was admitted to our hospital with a history of chest pain for 1 month. Computerized tomography of the thorax showed a left paracardiac mass adjacent to the aortic arch, left main pulmonary artery and pericardium. Total surgical excision could not be performed, but several biopsies which were taken through a thoracotomy revealed yolk sac tumor of the mediastinum. Systemic chemotherapy was started, but no response was observed and the patient died after one course of chemotherapy. CONCLUSION: Nonteratomatous mediastinal germ cell tumors are virtually nonexistent in women. To our knowledge, this is the oldest female patient with a primary mediastinal germ cell tumor. Chemotherapy was not effective especially in an old patient. We suggest surgery should be considered as primary treatment modality if diagnosis is made at an early stage.

Aged↗

Testicular seminoma after the complete remission of extragonadal yolk sac tumor : a case report.

BACKGROUND: Between 2% and 5% of malignant germ-cell tumors in men arise at extragonadal sites. Of extragonadal germ cell tumors, testicular carcinoma in situ (CIS) are present in 31-42% of cases, and CIS are reported to have low sensitivity to chemotherapy in spite of the various morphology and to have a high likelihood of developing into testicular tumors. A testicular biopsy may thus be highly advisable when evaluating an extragonadal germ cell tumor. CASE PRESENTATION: A 36-year-old man was diagnosed as having an extragonadal non-seminomatous germ cell tumor, that was treated by cisplatin-based chemotherapy, leading to a complete remission. In the meantime, testicular tumors were not detected by means of ultrasonography. About 4 years later, a right testicular tumor was found, and orchiectomy was carried out. Microscopically, the tumor was composed of seminoma. CONCLUSIONS: We herein report a case of metachronous occurrence of an extragonadal and gonadal germ cell tumor. In the evaluation of an extragonadal germ cell tumor, a histological examination should be included since ultrasonography is not sufficient to detect CIS or minute lesions of the testis.

Adult↗

Infantile and adult testicular germ cell tumors. a different pathogenesis?

Most adult testicular germ cell tumors have a characteristic chromosomal abnormality that is an isochromosome 12p [i(12p)]. Furthermore, these tumors are characterized by a chromosome number in the triploid range and gains and losses of (parts of) specific chromosomes. Cytogenetic investigation of three cases of infantile testicular germ cell tumors, all diagnosed as yolk sac tumors, revealed highly abnormal karyotypes. We found one case to be diploid; the other two cases were in the hypertriploid/hypotetraploid range. Structural abnormalities of chromosomes 1, 3, and 6 were recurrent and no i(12p) was found. Our results, together with data from the literature, suggest that infantile and adult testicular germ cell tumors have a different origin and pathogenetic pathway. Aberrations of chromosomes 1, 3, and 6 may play an important role in the pathogenesis of infantile testicular yolk sac tumors.

Adult↗

[Three cases of resected primary mediastinal yolk sac tumor following six courses of bleomycin, etoposide and cisplatin (BEP) combination chemotherapy].

We experienced three cases of primary mediastinal yolk sac tumor which were resected after 6 courses of BEP chemotherapy with G-CSF support. All cases had high levels of AFP. CT scan revealed an anterior mediastinal tumor infiltrating the surrounding tissue in all cases, and multiple pulmonary nodules in one case. The serum AFP level decreased markedly, but did not return to normal after the chemotherapy was completed. The markedly decreased mediastinal tumor masses were removed with en-bloc resection of the lung and pericardium. Viable tumor cells were not present in the resected tumors. These three cases remain free of disease at present, 3 years, 9 months and 5 months after operation.

Adult↗

Vaginal yolk sac tumor in a nine-month-old female child.

A case of yolk sac tumor in a nine-month-old girl is presented. Vaginal hemorrhage was observed during the patient's check-up at the Bjelovar Children's Outpatient Clinic. The patient was referred to the Department of Pediatrics at the Zagreb University Hospital, where an endodermal tumor of the vaginal yolk sac was diagnosed. The patient underwent surgery, followed by chemotherapy. She is now 3.5 years old, in good general health and under regular control.

Endodermal Sinus Tumor↗

Cytopathology of uncommon malignant renal neoplasms in the pediatric age group.

Malignant renal neoplasms are common solid tumors in pediatric oncology practice. These include the common Wilms' tumor/nephroblastoma and the uncommon neoplasms such as clear-cell sarcoma of the kidney (CCSK), rhabdoid tumor, renal-cell carcinoma, and others. The aim of this study was to describe in detail the cytopathological features of the histopathologically proven uncommon pediatric renal tumors. Aspirates from Wilms' tumor, which are mesenchyme predominant, show clusters of spindle cells associated with the matrix material. Evidence of rhabdomyoblastic differentiation may be present. CCSK, classic subtype, is characterized by round to oval cells arranged perivascularly and also in sheets and clusters intimately associated with a metachromatic matrix mucopolysaccharide material better appreciated in May-Grunwald-Giemsa (MGG)-stained smears. The cells also have more abundant cytoplasm and may show nuclear grooves. Spindle-cell pattern of CCSK is difficult to diagnose on aspiration cytology. Renal-cell carcinoma of childhood shows similar cytological features as its adult counterpart. Rhabdoid tumor of the kidney is characterized by a monomorphic population of cells with abundant cytoplasm, eccentric nuclei with prominent nucleoli. Intrarenal yolk sac tumor is a rare neoplasm and shows severely pleomorphic cells on aspiration. Awareness of these entities is important for the practicing cytopathologist. Further, non-Wilms' renal malignant neoplasms must be distinguished from the common Wilms' tumor so that appropriate chemotherapy protocols may be instituted in cases where the tumor is in an advanced stage of malignancy.

Adolescent↗

[Yolk sac tumor--case report and literature review].

Yolk sac tumor is the rare neoplasm, detected mostly in girls and young women above 30 yr. It is characterized by the high malignancy and short survival rate. We described the case of yolk sac tumor detected in 12-yrs old girl treated by combined method (nonradical tumor resection, chemotherapy, hysterectomy, bilateral adnexectomy and chemotherapy according to SFOP) and with the complete remission more than 1 year after the treatment was completed.

Antineoplastic Combined Chemotherapy Protocols↗

Indications for hemiscrotectomy in the management of genitourinary tumors in children.

In the last 30 years at St Jude Children's Research Hospital, 10 boys have undergone hemiscrotectomy for primary testicular tumors or paratesticular rhabdomyosarcoma. Indications for this procedure were scrotal contamination and positive or uncertain surgical margins. Residual tumor was found in only one specimen. There were four complications in the study group. Two patients had complications directly attributable to the hemiscrotectomy, and in one patient this caused a delay in treatment. Preoperative imaging was performed in three patients. This has not been helpful in evaluating residual masses and is not indicated. Based on the authors' experience and a review of the literature, there are a number of indications for hemiscrotectomy in the management of genitourinary tumors in children. Controversies regarding some of these indications should be discussed with the families of these patients during preoperative counseling.

Adolescent↗

Ovarian endometrioid tumors with yolk sac tumor component, an unusual form of ovarian neoplasm. Analysis of six cases.

The clinical, morphological, and immunohistochemical findings in six cases of ovarian endometrioid tumors (five endometrioid carcinomas and one carcinosarcoma) with a yolk sac tumor (YST) component are described. The age of the patients ranged from 31 to 73 years (average, 53), and only two patients were premenopausal. Two cases were stage Ia tumors, three stage III, and one stage IV. A substantial postoperative elevation of alpha-fetoprotein (AFP) was seen in two patients and a mild increase in another two. All six patients had surgery and postoperative cisplatin-based chemotherapy regimens, four of whom died of tumor 3 to 14 months after surgery without response to treatment. Only a stage Ia patient is alive and well 1 year after surgery. The tumors were large (average, 17 cm). Benign endometrioid lesions were found in the homolateral ovary in two cases and in the contralateral ovary in another two. All cases had endometrioid ovarian carcinomas (EOC) of various types admixed with typical YST components. Immunohistochemically, EOC areas differed from YST in their positivity for OC 125, CA 19.9, and nuclear estrogen and progesterone receptors and in their negativity for AFP, which was conspicuously positive in the YST areas. The clinicopathological profile of ovarian endometrioid tumors with YST also differs from that of YST in that it occurs in the same age range as EOC, it shows coexistence of benign endometrioid lesions, and it has a poor response to chemotherapy. The histological pattern in transitional areas may be difficult to differentiate from "endometrioid-like" (enteroblastic) YST and clear cell tumors. Ovarian endometrioid tumors with YST component should be considered a variant of endometrial carcinoma. Its recognition is necessary in view of its unusually aggressive behavior and poor prognosis.

Adult↗

Yolk sac tumor with a unique uniform hepatoid pattern histology.

BACKGROUND: Yolk sac tumors (YST) exhibit several different histological subtypes and the mechanisms of cellular differentiation and prognosis of each subtype remain unknown. RESULTS: We report two infants with sacrococcygeal YST; one represented a typical histological subtype and the other showed a hepatoid subtype with uniform histology. The isoform of alpha-fetoprotein (AFP) in the patient with the hepatoid pattern was examined by lectin-affinity immunoelectrophoresis and represented as a YST, but not hepatocellular, subtype. The patient with typical YST responded well to VAB-6 combination chemotherapy. However, this regimen was only partially effective to the patient with the pure hepatoid histological subtype, and an etoposide with ifosfamide and cisplatin (VIP) regimen as a salvage chemotherapy combined with complete tumor resection was useful to achieve complete remission (CR). Both of the patients have been in CR for more than four years.

Antineoplastic Combined Chemotherapy Protocols↗

Yolk sac tumor of the vulva: a case report with long-term disease-free survival.

BACKGROUND: Yolk sac tumor (YST) of the vulva is extremely rare. Seven cases of vulvar YST have been reported to the literature. Due to the rarity of tumors, the appropriate choice of treatment may remain unclear. CASE: A 30-year-old woman presented with a 3.5-cm right labial mass. Excisional biopsy showed YST with predominant solid pattern. Three weeks after excision, right inguinal lymph node biopsy revealed metastatic tumor. The serum alpha-fetoprotein (AFP) was not elevated. Cisplatin-based chemotherapy was administered, followed by pelvic and groin irradiation. The patient was free of disease 90 months after the diagnosis. CONCLUSION: Local excision of tumor with adjuvant cisplatin-based chemotherapy can be justified for vulvar YST. Inguinal lymphadenectomy is recommended because metastasis may occur early. Adjuvant radiation therapy may help to control the disease. Tumor size of 5 cm or less may be a favorable prognostic factor. Serum AFP level may not be a sensitive marker for follow-up of vulvar YST.

Adult↗

[Two cases of testicular tumor with brain metastasis].

During the last 8 years, 70 cases of testicular tumor were treated in our department. Of them 2 cases had brain metastasis. Case 1; A 37-year-old male was admitted with the chief complaint of cough. Retroperitoneal lymph node and lung metastases were discovered (stage IIIB, pT4aN1M1). Resected right testis was diagnosed as embryonal carcinoma, teratoma and STGC histopathologically. After 3 courses of PVB (cisplatinum, vincristine, bleomycin) chemotherapy right hemiplegia occurred and computerized tomographic (CT) scan revealed brain metastasis. His general condition degraded rapidly and he died of brain herniation 3 months after orchiectomy. Case 2; A 32-year-old male was admitted because of right testicular swelling and lung metastases (stage IIIB, pTiN0M1). Pathological examination revealed embryonal carcinoma, yolk sac tumor and teratoma. After 4 courses of chemotherapy with cisplatinum (CDDP), vincristine, methotrexate, peplomycin, and etoposide all lung metastases were disappeared. A few months later left hemiplegia by brain metastasis appeared suddenly. Four additional courses of high dose chemotherapy and resection of brain metastasis was performed. Complete remission continued for 13 months. The prognosis of testicular tumor with brain metastasis was very poor. During the last 8 years, 21 cases of testicular tumor with brain metastasis were reported in the Japanese literature. A follow-up study of prognosis in the literature was performed and discussed.

Adult↗

Supernumerary ovary found by ultrasonogram and FSH measurement after an extensive operation for a yolk sac tumor of the ovary.

A rare case of supernumerary ovary found by a transvaginal ultrasonogram and follicle-stimulating hormone (FSH) measurement is presented. The patient was a 32-year-old female who underwent an extensive operation for a yolk sac tumor of the ovary. An asymptomatic cystic tumor was found during follow-up. There was no evidence of recurrence or metastasis of the yolk sac tumor. Although histological confirmation was not possible because the patient refused removal of the mass, a diagnosis of supernumerary ovary was made because changes in the shape of the cystic mass completely correlated with changes in the serum FSH level.

Adult↗