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Query: UMLS:C0677930 (primary tumor)
 20,210 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Needle core biopsies (NCB) are widely used in adults but are less often used for the evaluation of pediatric tumors. To determine the diagnostic utility of NCB for pediatric tumors, we performed a retrospective analysis. Fifty NCB of masses from 1992 to 1998, subsequent pathologic specimens, and medical records were reviewed. All patients were less than 21 years of age. Of the NCB 78% (39/50) were diagnostic of a neoplasm, 8% (4/50) were nondiagnostic in cases where a tumor was subsequently diagnosed, and 14% (7/50) revealed inflammatory or reactive lesions, with no subsequent diagnosis of a neoplasm according to medical record review. In cases in which a neoplasm was present, NCB was diagnostic in 91% (39/43). For cases in which there was a previous diagnosis of a tumor, 100% (9/9) of NCB were diagnostic of a recurrence or metastasis. In cases of NCB for primary tumor diagnosis, 88% (30/34) were diagnostic. The most common problems encountered were related to specimen adequacy, such as insufficient tissue, crush artifact, and tumor necrosis. Tumor diagnoses were as follows: primitive neuroectodermal tumor (PNET)/Ewing sarcoma (12), malignant lymphoma/Hodgkin's disease (8), rhabdomyosarcoma (4), germ cell tumor (3), Wilms' tumor (3), neuroblastoma (1), sarcoma, not otherwise specified (4), and other neoplasms (8). There were no complications of the procedure. NCB of pediatric tumors is an effective diagnostic tool and can be used to obtain diagnostic material quickly and safely. NCB was diagnostic in 90% of cases in this series. When NCB provide sufficient material for immunohistochemical, cytogenetic, flow cytometric, and other ancillary studies, the diagnostic efficacy is enhanced. The major limitations in this series were related to sampling problems and specimen adequacy for comprehensive pathologic evaluation.
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PMID:Pediatric core needle biopsy: strengths and limitations in evaluation of masses. 1120 Apr 90

The outcome of 31 patients with malignant ovarian germ cell tumors treated by surgery and a medium dose etoposide containing short chemotherapy regimen between 1988 and 1997 is reported. Of the 31 patients, 16 (51.6%) had malignant teratomas, 8 (25.8%) had dysgerminomas, 6 (19%) endodermal sinus tumors and one (3.2%) mixed germ cell tumor. Twenty-four (77.4%) patients were at FIGO stage I (of which 18 were stage IA), 2 (6.5%) at stage II, 4 (12.9%) at stage III and 1 (3.2%) at stage IV. Twenty-five (80.6%) patients underwent conservative surgery, 1 (3.2%) underwent bilateral salpingo-oophorectomy and 4 (12.9%) had total hysterectomy with bilateral salpingo-oophorectomy and omentectomy. One (3.2%) patient refused definitive treatment. Three patients with stage IA grade 1 immature teratomas were not treated with adjuvant chemotherapy and one patient with a stage IA dysgerminoma refused chemotherapy. Two patients with endodermal sinus tumor returned to their countries of origin after surgery. Twenty-five patients received bleomycin, etoposide, and cisplatin (BEP) regimen with etoposide dosage fixed at 120 mg/m2 on day 1 and day 2, bleomycin 15 mg intravenous bolus on days 1 and 2 and cisplatin 100 mg/m2 on day 1. Chemotherapy was administered at four weekly intervals for 4 cycles or until complete response was achieved. The median number of cycles of chemotherapy was four (range 3-6) for stage I, 6 (range 4-7) for stage II and 5 (range 5-6) for stage III tumors. Of the entire cohort of 29 patients analyzed, the median follow up period was 5 years. One patient died from stage IIIC endodermal sinus tumor and one patient had persistent teratoma in the lungs. The overall disease free survival control rate was 93.1%. There were three cases of the growing teratoma syndrome involving the liver, abdominal peritoneum, and the pelvis, respectively. No mortality resulted from the growing teratomas. No pulmonary complications, secondary primary tumor or leukemia was detected. Menstrual function returned in all patients with fertility-preserving surgery and one pregnancy occurred. This interesting data suggest that a medium dose 2-day BEP postsurgical adjuvant chemotherapy regimen is effective and superior to expectant treatment of malignant ovarian germ cell tumors. This report, however, should be viewed as a pilot study. The result indicates that a prospective randomised controlled trial to demonstrate equivalence of this regimen with the standard BEP regimen is warranted.
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PMID:Experience of a 2-day BEP regimen in postsurgical adjuvant chemotherapy of ovarian germ cell tumors. 1124 Jun 46

A 63-year-old man was admitted to our hospital for complaints of a painless knot in his right testicle. The patient underwent orchiectomy for suspicion of malignancy. Pathologic examination detected a firm, circumscribed mass that consisted histologically of noncohesive, large, undifferentiated tumor cells diffusely infiltrating the testicular interstice. Because of the patient's advanced age and the structure of the neoplastic cells, the differential diagnosis favored a lymphoma over a malignant germ cell tumor but also included a dedifferentiated metastatic neoplasm. Immunohistochemistry was tailored accordingly and was completed using three successive panels of antibodies. Immunostaining with the first panel of antibodies directed against leukocyte epitopes (CD45, CD20, and CD3) remained negative and made a lymphoma unlikely. The subsequent panel (cytokeratin AE1/3, cytokeratin 18, and HMB-45) helped to rule out a malignant melanoma and aided to settle the diagnosis of a metastatic carcinoma. The reaction pattern of the last panel of antibodies pointed to a pulmonary origin of the putative primary tumor (PSA-negative, TTF-1-positive, and CK20-negative). The diagnosis of a metastasized poorly differentiated adenocarcinoma of the lung was confirmed by autopsy 5 months later. This case represents the extremely rare occurrence of a testicular metastasis as a primary manifestation of an occult neoplasm and shows the usefulness of an integrated site-specific clinicomorphologic approach that should precede and guide the choice of diagnostic immunoreagents.
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PMID:Immunohistochemical assessment of a testicular tumor in a 63-year-old patient: proposal for an integrated clinicopathologic approach. 1261 Mar 64

An unselected population of 635 consecutive extragonadal GCT patients (EGCT) treated between 1975 through 1996 at 11 cancer centers was retrospectively evaluated for clinical prognosis and biological features of this disease. Five hundred twenty-four patients (83%) had a nonseminomatous GCT, and 104 patients (16%) a seminomatous histology; 341 (54%) patients had a primary mediastinal EGCT, and 283 patients (45%) a retroperitoneal EGCT. Following platinum based induction chemotherapy+/-secondary surgery, 141 patients (49%) with mediastinal nonseminomas (median follow up period: 19 months) and 144 patients (63%) with retroperitoneal nonseminoma (median follow up period: 29 months) are alive [p=0.0006]. In contrast, the overall survival rate for patients with seminomatous EGCT is 88% with no difference between patients with mediastinal or retroperitoneal tumor location (median follow up period: 49 months). Multivariate analysis revealed nonseminomatous histology, the presence of non-pulmonary visceral metastases, primary mediastinal GCT location, and elevated beta-HCG as independent prognostic factors for shorter survival. Sixteen patients (4.1%) developed a metachronous testicular cancer despite the use of platinum based chemotherapy. The cumulative risk of developing a MTC 10-years after a diagnosis of EGCT was 10.3% (95% CI=4.9 to 15.6%), but higher among patients with nonseminomatous EGCT (14.3%; 95% CI=6.7 to 21.9%) or retroperitoneal EGCT location (14.2%; 95% CI=5.6 to 22.8%) than among patients with seminomatous EGCT (1.4%; 95% CI=0.0 to 4.2) or mediastinal EGCT location (6.2%; 95% CI=0.1 to 12.2). After a median follow-up of 51 months (range=1 to 154 months), all 16 MTC patients were alive without disease. Patients with pure seminomatous EGCT histology have a long term chance of cure of almost 90% irrespective of the primary tumor site. Patients with mediastinal nonseminomas have a five-years survival rate of 45%. This outcome is clearly inferior compared to patients with nonseminomatous retroperitoneal primaries who have a five-year survival rate of 62%.
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PMID:Extragonadal germ cell tumors: relation to testicular neoplasia and management options. 1275 35

Two cases of germ cell neoplasm retrospectively considered to have been of testicular origin are reported. Case 1. A 19-year-old male with brain, liver and retroperitoneal tumors was diagnosed with yolk sac tumor by retroperitoneal tumor biopsy. After multidisciplinary treatment, a region of calcification was detected in the left testis on scrotal sonography and left high inguinal orchiectomy was performed. Case 2. A 57-year-old male with neck, lung and retroperitoneal tumors was diagnosed with yolk sac tumor by supraclavicular biopsy. From initial examination, scrotal sonography revealed a small calcified lesion in the right testis. After chemotherapy, high inguinal orchiectomy and retroperitoneal lymphadenectomy were simultaneously performed. Pathologic evaluation of these testicular specimens revealed calcification and a fibrous scar in correspondence with the clinical diagnosis. These changes were considered as scars of the primary testicular tumor due to burned-out tumor or the result of reaction to chemotherapy. Since a primary tumor of testicular origin may exist in the extragonadal germ cell tumor, it is important to examine the intrascrotal contents in detail in the case of so-called extragonadal germ cell tumors with palpably normal testes. In such cases, there are two possible conditions, an occult testicular tumor and a burned-out testicular tumor. We briefly reviewed 42 such cases in the Japanese literature. It appears that there are very few true extragonadal germ cell tumors, and that the possibility of primary testicular origin metastasizing from viable occult testicular tumor or burned-out testicular tumor with spontaneous regression is high in retroperitoneal germ cell tumors.
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PMID:[Retroperitoneal germ cell tumor with testicular calcification indicating tiny testicular origin: consideration of the origin of retroperitoneal germ cell tumors: report of two cases]. 1282 60

We have performed comparative genomic hybridization on 12 testicular germ cell tumor (TGCT) cell lines and one paraffin-embedded surgical specimen to identify and characterize genome-wide gains and losses of chromosomes in these specimens. All specimens demonstrated overrepresentation of 12p. Other significant chromosomal gains, apart from 12p, included the X chromosome and chromosome arms 1q and 20q. Chromosomal losses were observed for chromosomes 4 and 18 and chromosome arms 2q, 9q, and 13q. Genomic differences were observed between an embryonal carcinoma component of a mixed tumor, 833K, and its cisplastin-resistant derivative line, 64CP, including losses of 6q23 approximately qter and 9p22 approximately q21. Five lines also demonstrated gain of 12p and additional 12p12 approximately p13 material. Similarly, two lines demonstrated gain of 12p and additional 12p11.2 approximately p12 material. The data supports the consistent gain of 12p in adult TGCT cell lines and additional regional amplification of 12p in some lines. This regional amplification has been observed in both primary tumor specimens and TGCT cell lines and may support a hypothesis that at least two different regions of 12p, one proximal and one distal, harbor genes important for the pathogenesis of testicular germ cell neoplasia.
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PMID:Characterization of gains, losses, and regional amplification in testicular germ cell tumor cell lines by comparative genomic hybridization. 1469 36

We describe the clinicopathologic findings in a so far unrecognized thymic tumor. The tumor occurred in a 70-year-old woman with respiratory distress but neither myasthenia gravis nor other symptoms. Metastases or another primary tumor were absent. The well-circumscribed neoplasm was located in the thymic region, measured 18 x 12 x 8 cm, and showed a homogeneous, tan-colored, soft cut surface. By histology, the tumor lacked a true capsule and a lobular growth pattern, was almost devoid of stroma, and infiltrated among remnant thymus lobules. The polygonal tumor cells formed solid sheets, trabeculae, or occurred as single cells that resembled hepatocytes. Proliferative activity was low. Portal structures, sinuses, and bile were absent as were areas of conventional thymoma, adenocarcinoma, or germ cell tumor. The tumor expressed cytokeratins 7 and 19, alpha1-antitrypsin, alpha1-antichymotrypsin, and hep-Par-1. Alpha-fetoprotein (AFP), human beta-chorionic gonadotropin (beta-HCG), placental alkaline phosphatase, CD5, CD30, CD31, CD34, CD45, CD68, CD99, S-100, HMB45, desmin, actin, or neuroendocrine markers were not expressed, and intratumorous CD1a+ or TdT+ immature T cells were absent. AFP was repeatedly undetectable in the blood. Mediastinal tumor recurrence was detected 6 months after surgery. Following radiochemotherapy, the patient has remained free of disease for 26 months. We conclude that this tumor is a thymic carcinoma (WHO type C thymoma). A diagnosis of hepatoid yolk sack tumor appears unlikely considering absence of a bona fide germ cell component, lack of AFP expression, and the patient's female gender. Because of its morphologic and immunohistochemical features, we propose the term "hepatoid thymic carcinoma" for this new type of thymic carcinoma.
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PMID:Hepatoid thymic carcinoma: report of a case. 1504 16

Due to its unique biological behavior, late relapse (LR) of testicular cancer has recently been described as an own tumor entity. It is currently defined as tumor recurrence more than 2 years after complete remission following primary treatment including chemotherapy. The incidence ranges from 2 to 6%, with a median relapse-free interval of 5.4-7.1 years from the initial treatment. Although histology shows a germ cell tumor (GCT) origin, the clinical biology is different. The dominant characteristics are slow tumor growth and chemoresistance. Molecular analysis currently focuses on the mechanisms of drug resistance. The initial response to chemotherapy is less than 30% and in most cases complete surgical resection remains the only treatment option with favorable long-term results. The most common site of LR is the retroperitoneum, with undifferentiated cancer (yolk sac) being the most frequent histology. In most cases, patients have already undergone previous retroperitoneal surgery. The overall cure rate is only about 50%, hence, adequate treatment of the primary tumor is essential to prevent the development of LR.
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PMID:Late relapse of testicular cancer. 1506 70

We report a nephroblastoma arising in a germ cell tumor of testicular origin occurring in a 22-year-old man. Orchiectomy demonstrated a malignant mixed germ cell tumor composed of mature and immature teratoma with nephroblastoma and rhabdomyosarcoma. Following chemotherapy, the patient developed supraclavicular and retroperitoneal lymphadenopathy. Excision demonstrated metastatic teratoma at both sites. No recurrence was noted with 21 months of additional follow-up. Using tissue microdissection and loss of heterozygosity analysis, we investigated the clonality of the mature teratoma, immature teratoma, nephroblastoma, and rhabdomyosarcoma components of the primary tumor and of the metastatic mature teratoma at the two separate distant sites. Nine microsatellite polymorphic makers were used to examine the pattern of allelic loss in both primary and metastatic tumors. Loss of heterozygosity was found in 4 DNA loci, and the same pattern of allelic loss was demonstrated at all 4 loci in all of the different components of the primary tumor and the metastatic mature teratomas, supporting the germ cell tumor origin of the nephroblastoma component. Loss of heterozygosity on chromosome 17p13 (TP53) was detected in metastatic mature teratoma, but not in the primary tumor. Loss of heterozygosity was observed at 11p13, the locus of WT1 inactivation in patients genetically predisposed to nephroblastoma, and this loss may be an important genetic mechanism in nephroblastomatous differentiation of germ cell tumors. These data support a common clonal origin for nephroblastoma and the other germ cell tumor components.
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PMID:Nephroblastoma arising in a germ cell tumor of testicular origin. 1510 60

Advanced testicular germ cell tumors commonly involve cervical lymph nodes. In most circumstances, the diagnosis of germ cell tumor is established before the neck disease is noted. In rare cases, these tumors have been found along with cervical lymphadenopathy in patients with a previously undiagnosed primary tumor. In this article, we report the unusual case of a 71-year-old man whose metastatic seminoma initially manifested as an asymptomatic neck mass. This finding reinforces the need to include metastatic disease in the differential diagnosis of neck masses. Our discussion of this case focuses on the appropriate management of cervical metastases of germ cell tumors.
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PMID:Metastatic seminoma with cervical lymphadenopathy as the initial manifestation. 1519 84


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