Fludeoxyglucose F 18 positron emission tomography and computed tomography of a giant retroperitoneal schwannoma occurring in a patient with neurofibromatosis type 2.
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Adolescent↗
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Results for “Retroperitoneal Neoplasms”
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Diagnostic sonography in general surgery.
Eighty-seven patients who had sonographic examinations at the Orange County Medical Center were studied retrospectively to evaluate the usefulness and reliability of this noninvasive technique, and sonographic results were compared with findings at operation or findings made using other diagnostic methods. With a variety of lesions, the overall accuracy of diagnosis by sonography was 85%. In the remaining 15% of studies, false-positive or false-negative results were observed. The accuracy varied with the organ involved, the greatest being renal and hepatic masses. Ultrasound, when used in addition to careful surgical assessment of the patient, was found to be most useful in the diagnosis and follow-up of abdominal problems in surgical patients.
Abscess↗
Mesenteric cysts and intra-abdominal cystic lymphangiomas.
Although mesenteric cysts and intra-abdominal cystic lymphangiomas are uncommon and clinically confusing lesions, histologic and ultrastructural evidence suggests that they are pathologically distinct. Differentiation of these lesions is important since lymphangiomas may follow a proliferative and invasive course. Of 28 cases documented at laparotomy, histologically eight patients (29%) had cystic lymphangiomas and 20 patients (71%) had mesenteric cysts. Lymphangioma was found to be exclusively a disease of childhood and young adulthood (mean age, 10 years); mesenteric cyst was found in all age groups (mean age, 44 years), and two thirds of these patients were over 40 years old. Patients with lymphangiomas more frequently were male (75% vs 30%), symptomatic (88% vs 35%), and had ascites (50% vs 0%) and larger lesions (mean, 8.8 vs 4.7 cm) when compared with patients with mesenteric cysts. Complete excision was possible in all but four patients, with no operative deaths and a postoperative complication rate of 7%. After a mean follow-up period of four years, there were no recurrences among 16 patients who had undergone complete excision.
Abdominal Neoplasms↗
Tissue distribution of mutant mitochondrial DNA in a patient with MERRF syndrome.
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Aged↗
Outcome prediction by molecular detection of minimal residual disease in bone marrow for advanced neuroblastoma.
BACKGROUND: We have determined whether sequential molecular detection of minimal residual disease (MRD) in bone marrow (BM) could predict the outcome of patients with advanced neuroblastoma (NB). PROCEDURE: Bone marrow samples from 19 patients over 12 months of age with stage 4 neuroblastoma were sequentially examined for tumor cell contamination by detecting tyrosine hydroxylase (TH) mRNA using reverse transcription-polymerase chain reaction (RT-PCR). All patients received repetitive multi-drug chemotherapy including cisplatin, cyclophosphamide or ifosphamide, adriamycin, and etoposide or vincristine. Seventeen patients received myeloablative therapy with hematopoietic stem cell transplantation after achieving complete remission. RESULTS: All but one patient were histologically positive for tumor cells in BM samples at diagnosis, and they became negative for tumor cells within 3 months histologically. By the RT-PCR analysis, all patients were positive for TH mRNA in BM samples at diagnosis, and they became negative for TH mRNA 1 to 13 months after the start of chemotherapy. Six patients whose BM samples became negative for TH mRNA within 4 months after the start of chemotherapy remained alive without evidence of disease (median 61 months, range 20-76). In contrast, 12 of 13 patients whose BM samples remained positive at that time developed relapse and 10 of them died of disease (median 24 months, range 13-43). There was a statistically significant difference in survival between the two groups (P < 0.05). No significant difference of clinical characteristics by the MRD positivity at 4 months after the start of chemotherapy. CONCLUSIONS: Persistence of MRD in BM at 4 months after the start of chemotherapy could predict poor prognosis in advanced neuroblastoma.
Adrenal Gland Neoplasms↗
Tumor hypoglycemia.
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Abdominal Neoplasms↗
Lymphoreticular sarcomas with primary involvement of Waldeyer's ring. Clinical evaluation of 225 cases.
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Biopsy↗
Occult seminoma of the testis masquerading as primary extragonadal germinal neoplasms.
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Abdominal Neoplasms↗
Ultrasonic imaging of neoplasms.
Reflected ultrasound scanning (echography) is rapidly developing into a prime tool for tumor diagnosis, outlining, and detection. The principles of echographic diagnosis of neoplasms, the accepted uses in detection and diagnosis, as well as some of the principles of internal and contour patterns are discussed. Echography is very helpful in determining the size, location, organ origin, internal characteristics, and course of neoplasms in the abdominal area, neck, and extremities. Echography is helpful in radiation therapy planning and is expected to assume an increasing role in the diagnosis and management of tumors.
Abdominal Neoplasms↗
Qualitative and quantitative analyses of human chorionic gonadotropin and its subunits produced by malignant tumors.
Human chorionic gonadotropin (hCG) and its subunits in plasma, urine, ascites, and tumor extracts from four patients with hCG-producing tumors (undifferentiated cell carcinoma and choriocarcinoma of the bladder, malignant teratoma of the retroperitoneum, and pancreatic carcinoma) were measured by the radioimmunoassays specific to each component. While both free alpha and beta subunits as well as the whole molecule of hCG were found in all these samples, the proportion of beta subunit was much higher in these tumor extracts than in the placental extracts. Since the alpha and beta subunits are known to be translated from separate mRNAs, such increase of subunit is probably due to the predominant increase of mRNA encoding beta subunit of hCG in the hCG-producing tumors. Gel filtration of the extracts of these four tumors on Sephadex G-100 and G-150 columns demonstrated a heterogeneity of hCG, alpha, and beta subunits in these tumor extracts, and the elution profiles of plasma, urine and tumor extracts were slightly different. Lower molecular weight forms of hCG and its beta subunit were present in all urine samples. The significance of these heterogeneities remains to be elucidated.
Adult↗
The development of non-germ cell malignancies within germ cell tumors. A clinicopathologic study of 11 cases.
Eleven male patients had germ cell tumors of the testis (7), mediastinum (3), or retroperitoneum (1) in which non-germ cell malignancies developed. Such malignant non-germ cell elements were present in the primary excisions of five patients and were subsequently found in additional resected tissue in 10 of 11 patients. In the patients who had multiple pathology specimens examined, a progression from atypia to predominant non-germ cell malignancy was often found. The authors believe these malignant elements arose within teratomatous foci, since eight of nine cases had teratoma in the primary tumor, and teratoma was found in subsequently resected tissue in one additional case. Cisplatin therapy frequently "unmasked" the non-germ cell malignant elements by destroying the more chemosensitive germ cell cancers. The prognosis was worst for five patients who developed progressive embryonal rhabdomyosarcoma: two of these patients died of local spread of tumor, whereas a third died of metastatic sarcoma. Only one patient, who had total surgical excision of rhabdomyosarcoma, survived. Other forms of sarcoma that developed within germ cell tumors did not appear to adversely affect the prognosis beyond that of teratoma. It is currently recommended, when feasible, that patients with teratoma and sarcoma undergo total surgical excision. Further treatment with cisplatin regimens, after eradication of the germ cell component, has not been helpful. The role of other forms of chemotherapy remains speculative.
Adolescent↗
Ovarian sex cord tumor with annular tubules.
A pathologic study was done on four cases of ovarian sex cord tumor with annular tubules. All four tumors occurred in young women (11-24 years of age) and were not associated with the Peutz-Jeghers syndrome. Two patients had evidence of hyperestrinism. One patient who had metastasis to the retroperitoneum, left supraclavicular lymph node, and liver confirmed the malignant potential of this tumor. Gross examination revealed tumors that were solid, yellowish, and unilateral, with varying degrees of cystic degeneration. Microscopic examination showed simple or complex annular tubules with prominent basement membranes. Many tumor cells contained lipid in the cytoplasm. Ultrastructural study showed Charcot-Bottcher filaments in all four cases, indicating Sertoli cell differentiation. True lumens and microvilli were identified in one case. The classification of the sex cord tumor with annular tubules as a Sertoli cell tumor, annular tubular type was proposed on the basis of ultrastructural findings.
Adolescent↗
Cellular schwannoma. A clinicopathologic study of 57 patients and 58 tumors.
The cellular schwannoma is a variety of schwannoma with a predominantly cellular growth but no Verocay bodies. Because doubt has been raised about the original assessment of this tumor as benign, the clinical and pathologic characteristics of 58 cellular schwannomas from 57 patients were reviewed. The patients were most often middle aged (63% were female), and their tumors most commonly were painless masses with a predilection for the paravertebral region of the retroperitoneum, pelvis, and mediastinum. Most tumors were solitary and encapsulated, and an associated nerve was identified for 43% of the cases. Electron microscopic and immunohistochemistry studies confirmed the tumor's Schwann cell nature. Worrisome features such as bone erosion, hypercellularity, foci of necrosis (four tumors), hyperchromasia, nuclear pleomorphism, and the presence of mitotic figures led to a malignant diagnosis for 28% of the cases. Treatment in all but one case was surgical excision. Two patients also received radiation therapy and chemotherapy. Follow-up of from 1 year to 24 years, 7 months (median of 6 years and mean of 7 years) for 61% (35 cases) of the cases reveals three patients with a local recurrence but no cases in which the tumor metastasized or the patient died of the tumor. Awareness of this tumor type is important so that the surgeon will avoid unnecessary sacrifice of functionally important nerves attached to these tumors and so that needless adjuvant radiation and chemotherapy will not be instituted.
Adolescent↗
Molecular phenotype of a pediatric small round cell tumor.
Molecular probes were used to characterize an unusual small round cell abdominal tumor arising from the fallopian tube of a 15-year-old girl. DNA and RNA extracted from the tumor and adjacent normal tissue was subjected to Southern and Northern blot analysis using a variety of different probes. N-myc oncogene RNA was greatly expressed in the tumor, but was not expressed in normal tissue or amplified in chromosomal DNA. Insulin-like growth factor II RNA was similarly overexpressed in the neoplasm, but not in normal tissue. While histopathologic studies could not distinguish between a neuroectodermal neoplasm and Wilms' tumor, electron microscopy and the pattern of gene expression was most consistent with Wilms' tumor.
Adolescent↗
Association of expression between N-myc gene and major histocompatibility complex class I gene in surgically resected human neuroblastoma.
Amplification of the N-myc gene in neuroblastoma correlates with advanced stage and poor prognosis. Association of the expression between N-myc and major histocompatibility complex (MHC) class I genes in 33 neuroblastomas obtained from Japanese children was investigated. Amplification of the N-myc gene was observed in two of five cases in Stage III, six of 11 cases in Stage IV, and one of five cases in Stage IV-S. In each case, the expression of N-myc gene was significantly increased. The expression was also increased in cases without amplification of the N-myc gene, the origin being from the suprarenal region. Expression of the MHC class I gene was significantly decreased in five of these nine with a high level of N-myc expression with amplification. These results suggest that the down-modulation of the MHC class I expression may be associated with the high level of expression and amplification of N-myc gene in the advanced stage of neuroblastoma.
Child↗
Primary extracranial rhabdoid tumors. Clinicopathologic features and response to ifosfamide.
BACKGROUND: Malignant rhabdoid tumor (MRT) is an aggressive, invariably lethal tumor that is resistant to multimodal therapy. METHODS: The authors reviewed the clinicopathologic features, treatment, and outcome of 13 children (7 boys and 6 girls) with diagnoses of primary extracranial MRT at St. Jude Children's Research Hospital between 1981 and 1990. RESULTS: The median age at diagnosis was 8 months (range, 10 weeks-18 years). Primary sites included the kidney (seven patients), liver (three patients), soft tissue of scapula, posterior mediastinum, and retroperitoneum. Seven patients had metastatic disease (lungs, six patients; cutaneous hemangioma, one patient). Ten patients had surgical resection of primary tumor (complete, nine patients; incomplete, one patient). Eleven patients had chemotherapy with multiple agents. Three of four chemotherapy responses observed were with regimens containing ifosfamide. Partial responses (PR, > 50% reduction in tumor size) were obtained in one patient who received single-agent ifosfamide during disease relapse (PR lasting 2 months), one patient who received a combination of ifosfamide, carboplatin, and etoposide at diagnosis (PR lasting 5 months), and one patient who was treated with bleomycin, cyclophosphamide, doxorubicin, and vincristine at diagnosis (PR lasting 5 months) and subsequently with ifosfamide in combination with carboplatin and etoposide during disease relapse (PR lasting 4 months). All patients died at a median period of 5 months (range, 0.5-30 months) after diagnosis. CONCLUSIONS: Based on this review, the authors recommend using ifosfamide alone or in combination with carboplatin and etoposide in front-line therapy for malignant rhabdoid tumor.
Adolescent↗
Results of a Phase I study utilizing monocyte-derived dendritic cells pulsed with tumor RNA in children with Stage 4 neuroblastoma.
BACKGROUND: A Phase I study of 11 pediatric patients with newly diagnosed, Stage 4 neuroblastoma was conducted using monocyte-derived dendritic cells (DC) pulsed with tumor RNA to produce antitumor vaccines (DC(RNA)). METHODS: Patients received two courses of induction with carboplatin followed by standard chemotherapy, surgery, radiation, high-dose therapy, stem cell rescue, and DC(RNA) vaccine therapy. RESULTS: The results showed that this method for producing and administering DC(RNA) from a single leukapheresis product was both feasible and safe in this pediatric neuroblastoma population. Two courses of carboplatin maintained lymphocyte counts at normal levels. However, immune function 6 weeks after high-dose chemotherapy and stem cell rescue and prior to receiving DC(RNA) was impaired in all patients tested. There was an alteration in the ratio of CD4-positive and CD80-positive T cells. CD4-positive cell numbers were below normal, whereas CD8-positive cell numbers were above normal for all patients. In addition, CD19-positive cell numbers were below normal for all but one patient. It was found that humoral responses to recall antigens (diphtheria and tetanus) and cellular responses to mitogen and recall antigens were below normal in most patients. Despite this, two of three patients tested showed a tumor-specific humoral immune response to DC(RNA). Among the patients who had measurable disease at the time of DC(RNA) vaccine, none showed any objective tumor response. CONCLUSIONS: DC(RNA) vaccines were both safe and feasible in children with Stage 4 neuroblastoma. Humoral responses to tumor were detected, although remained immunosuppressed at the time of administration, limiting efficacy.
Adrenal Gland Neoplasms↗
Prostatic adenocarcinoma metastases mimicking small cell carcinoma on fine-needle aspiration.
Prostate adenocarcinoma (PA) is known to metastasize widely to bone, lung, lymph nodes, and other sites. We have observed a rare, although distinctive, neuroendocrine (NE) cytomorphology of metastatic PA on fine-needle aspiration (FNA) that mimics small cell carcinoma (SCC). From a total of 117 cases, eight cases of metastatic PA diagnosed on FNA showed cytomorphologic features indistinguishable from SCC. All specimens were reviewed, along with immunoperoxidase (IPOX) studies using prostate specific (PSA, PSAP) and NE markers (synaptophysin, chromogranin, etc.). The patients ranged in age from 51-68 (mean age = 63). The PSA levels at the time of FNA ranged from <0.1 to 2,892 ng/ml (normal postprostatectomy <0.2 ng/ml). Sites of FNA included liver (two), soft tissue (five), and lymph node (one). FNA was performed from 11 mo to 6 yr after the initial diagnosis of the primary tumor. All primary PA were of high Gleason grade ranging from 7-9. None of the primary PA showed neuroendocrine morphology. Cytomorphologic characteristics observed on FNA included predominantly single cells with occasional sheets or loose cell aggregates. A predominant NE nuclear morphology was evident (i.e., hyperchromasia, fine dusty chromatin, inconspicuous nucleoli, nuclear molding, chromatinic crush artifact, karyorrhexis, mitoses, etc.), with none of the tumors displaying glandular formation. Taken together, these features gave these metastases a cytomorphology indistinguishable from SCC. IPOX studies revealed PSA-positivity (5/7), PSAP-positivity (4/7), and only focal NE markers positivity (3/6). Metastatic prostate carcinoma may rarely mimic a SCC (6.8% in this study). This often necessitates further patient workup to identify the primary source for the patient's metastasis, particularly if the patient has multiple lesions. An accurate diagnosis of these lesions as PA metastases is essential for effective, timely treatment and therapeutic design.
Adenocarcinoma↗