Synchronous Wilms Tumor and Fibrolamellar Hepatocellular Carcinoma: Report of a Case

2000 ◽  
Vol 3 (5) ◽  
pp. 492-496 ◽  
Author(s):  
Anirban Maitra ◽  
Dharamdas M. Ramnani ◽  
Linda R. Margraf ◽  
Adi F. Gazdar

Fibrolamellar hepatocellular carcinoma (FHCC) is a unique histologic variant of HCC that occurs in a younger subset of patients than classical HCC, and is associated with a better prognosis. Wilms tumor (WT) is a malignant embryonal neoplasm of the kidney and is one of the most common solid tumors of childhood, occurring at an estimated frequency of 1 in 8000 to 10,000 births. Although second malignant neoplasms (SMNs) following therapy for WTs have been reported in the liver, the coexistence of HCC and WT is extremely rare. We present the first report of a synchronous ana-plastic WT and FHCC in a previously healthy 4-year-old girl. Despite the presence of focal immunohistochemical positivity for p53 in the WT, molecular analysis failed to reveal a germline or somatic p53 mutation, and was inconclusive in establishing a clonal relation between the two tumors.

Cancer ◽  
1991 ◽  
Vol 67 (2) ◽  
pp. 342-344 ◽  
Author(s):  
Jeffrey J. Kovalic ◽  
Patrick R. M. Thomas ◽  
J. Bruce Beckwith ◽  
James H. Feusner ◽  
Patricia A. Norkool

PEDIATRICS ◽  
1980 ◽  
Vol 66 (1) ◽  
pp. 121-123
Author(s):  
Cirilo Sotelo-Avila ◽  
F. Gonzalez-Crussi ◽  
Kenneth A. Starling

Patients with incomplete forms of Beckwith-Wiedemann syndrome (BWS) associated with solid tumors have been described in the literature, but they have not been sufficiently appreciated. A review of reports of children with asymmetry and intra-abdominal malignancy disclosed 12 patients who might represent BWS either incompletely manifested or insufficiently described1-12 (Table). Seven of these patients had nephroblastomas,1-7 two had adrenal cortical carcinomas,8,9 two had hepatoblastomas,10,11 and one had hepatocellular carcinoma.12 Three of these children had, or subsequently developed, a second tumor. One had nephroblastoma and adrenal adenoma1 another a bilateral cortical carcinoma metastatic to the liver and a lumbar lipoma9; the last one a nephroblastoma followed by the appearance of adrenal cortical carcinoma and breast fibroadenoma.7


1985 ◽  
Vol 3 (4) ◽  
pp. 532-538 ◽  
Author(s):  
A T Meadows ◽  
E Baum ◽  
F Fossati-Bellani ◽  
D Green ◽  
R D Jenkin ◽  
...  

This paper presents an update from the Late Effects Study Group on 292 cases of second malignant neoplasms (SMN) occurring in individuals who were diagnosed with their first neoplasm in childhood. Data are presented regarding the types of first and second neoplasm, the therapy administered, and the predisposing factors. Of the 292 cases (308 SMN), the most common primary was retinoblastoma followed by Hodgkin's disease, soft-tissue sarcomas, and Wilms' tumor. This is not similar to the relative frequency of these cancers in children but rather reflects specific risk factors. Bone sarcomas were the most common SMN among the 208 SMN developing in previously irradiated sites while acute leukemia was the most common SMN unassociated with radiation. Known predisposing conditions to cancer were present in 73 cases; retinoblastoma was the most common of these, followed by neurofibromatosis. There were ten patients with three and three patients with four malignant neoplasms. In 14 patients, the cause of SMN was not suggested by known risk factors as these patients had negative family histories and received no radiation or chemotherapy. We note, therefore, that although most cases of SMN in survivors of childhood cancer can be attributed to radiation, genetic disease, chemotherapy, or combinations of these, unrecognized predisposition or chance may also play a role.


1988 ◽  
Vol 80 (8) ◽  
pp. 592-595 ◽  
Author(s):  
N. E. Breslow ◽  
P. A. Norkool ◽  
A. Olshan ◽  
A. Evans ◽  
G. J. D'Angio

2009 ◽  
Vol 27 (15_suppl) ◽  
pp. 10003-10003
Author(s):  
M. M. O'Brien ◽  
S. S. Donaldson ◽  
A. S. Whittemore ◽  
M. P. Link

10003 Background: Second malignant neoplasms (SMN) are a known complication of Hodgkin disease (HD) treatment. While it is theorized that lower radiation doses may be associated with lower SMN risk, long-term follow-up of children treated with low-dose radiation is lacking. We report the occurrence of SMN among pediatric HD survivors treated at Stanford with chemotherapy and low-dose radiation from 1970 to 1990. Methods: Patients received 6 cycles of MOPP (mechlorethamine, vincristine, prednisone, procarbazine) with 15–25.5 Gy radiation ± 10 Gy boost or 3 MOPP and 3 ABVD cycles (doxorubicin, bleomycin, vinblastine, dacarbazine) with 15 Gy radiation ± 10 Gy boost. Follow-up was obtained through 9/1/2007. Standardized incidence ratios (SIRs) and absolute excess risk (AER) for SMN were calculated using the SEER9 database (1973–2004). Cumulative SMN incidence was determined by the Kaplan-Meier method. Univariate associations were evaluated with the chi-square test or t-test. Multivariate analysis was performed with Cox proportional hazards regression using chronological age as the time scale. Results: One hundred ten of 112 patients achieved remission; median follow-up is 20.6 years. Four patients developed secondary leukemia. Fifteen patients developed 17 secondary solid tumors (5 thyroid carcinomas, 6 breast carcinomas, 4 sarcomas, 1 bladder paraganglioma, 1 melanoma) at a median of 15.4 years. All solid tumors except the melanoma occurred within or at the margin of radiation fields, ranging in dose from 15–26.5 Gy. There were no cases of lung, gastric, or colorectal carcinoma. Cumulative incidence of any SMN is 17% (95%CI 10.5–26.7) at 20 years following HD diagnosis. The SIR for any SMN is 22.9 (95%CI 14.2–35) with an AER of 93.7 cases per 10,000 person-years. In univariate analysis, older age at HD diagnosis (>11 years) and female gender were associated with SMN (p<0.05). In multivariate analysis, there were no statistically significant associations. Conclusions: The incidence of SMN in pediatric HD survivors is elevated following treatment with chemotherapy and low-dose radiation. Sarcomas, breast, and thyroid carcinomas occurred with similar frequency and latency as found in studies of HD survivors who received high-dose radiation. No significant financial relationships to disclose.


2015 ◽  
Vol 62 (7) ◽  
pp. 1259-1264 ◽  
Author(s):  
Jean S. Lee ◽  
Benjamin Padilla ◽  
Steven G. DuBois ◽  
Aris Oates ◽  
John Boscardin ◽  
...  

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