John J. Doski

Parathyroid carcinoma in more than 1,000 patients: A population-level analysis.

BACKGROUND Parathyroid carcinoma (PC) is a rare malignancy with a moderate prognosis. The staging system, prognostic indicators, and optimal surgical management are still under debate. This large cohort explores prognostic factors for PC. METHODS 1,022 cases of PC in the 1998-2011 National Cancer Data Base that underwent surgery were examined for predictors of lower overall survival (OS) and relative risk (RR) of death at 5 years. RESULTS The 5-year OS was 81.1% in 528 patients with ≥ 60 months of follow-up. The overall cohort was mainly non-Hispanic (96.5%), white (77.4%), and insured (94.3%), with a median age of 57 years. Mean OS was lower and RR of death greater in older (P < .001), black (P = .007) patients with a secondary malignancy (P = .015) and ≥ 2 comorbidities (P = .005), whose surgical specimen had positive surgical margins (P = .026) or positive lymph nodes (P < .001). Multivariate cox regression demonstrated that positive lymph nodes (hazard ratio [HR], 6.47; 95% CI, 1.81-23.11) and older age (HR, 2.35; 95% CI, 1.25-4.43) were associated with lower OS. CONCLUSION PC is a rare malignancy with a 5-year OS of 81.1%. Positive lymph nodes and older age predict lower OS and an increased risk of death.

Colorectal carcinoma in pediatric patients: A comparison with adult tumors, treatment and outcomes from the National Cancer Database

BACKGROUND Pediatric colorectal cancer (CRC) is rare. Comparison with adult CRC tumors, management, and outcomes may identify opportunities for improvement in pediatric CRC care. STUDY DESIGN CRC patients in the National Cancer Data Base from 1998 to 2011, were grouped into Pediatric (≤21years), early onset adult (22-50) and older adult (>50) patients. Groups were compared with χ(2) and survival analysis. RESULTS A total of 918 pediatric (Ped), 157,779 early onset adult (EA), and 1,304,085 older adults (OA) were identified (p<0.01 for all comparisons). Patients ≤50 presented more frequently with stage 3 and 4 disease (Ped: 62.0%, EA: 49.7%, OA: 37.3%) and rectal cancer (Ped: 23.6%, EA: 27.5%, OA: 19.2%). Pediatric histology was more likely signet ring, mucinous, and poorly differentiated. Initial treatment was usually surgery, but patients ≤50 were more likely to have radiation (Ped: 15.1%, EA: 18.6%, and OA: 9.2%) and chemotherapy (Ped: 42.0%, EA: 38.2%, and OA: 22.7%). Children and older adults showed poorer overall survival at 5years when compared to early onset adults. Adjusting for covariates, age ≤21 was a significant predictor of mortality for colon and rectal cancers (colon HR: 1.22, rectal HR: 1.69). CONCLUSIONS This is the largest cohort of pediatric CRC patients, revealing more aggressive tumor histology and behavior in children, particularly in rectal cancer. Despite standard oncologic treatment, age ≤21 was a significant predictor of mortality. This is likely owing to worse tumor biology rather than treatment disparities and may signal the need for different therapeutic strategies.

Primary malignant pulmonary tumors in children: a review of the national cancer data base

BACKGROUND The purpose is to delineate the clinical and pathological characteristics of rare primary malignant pulmonary tumors in children. METHODS Utilizing the National Cancer Data Base (NCDB), we analyzed all children (≤ 18 years) with a primary malignant pulmonary tumor from 1998 to 2011 to identify factors associated with better survival. RESULTS Of 211 children identified, the most common histology was carcinoid tumor (n=133, 63%) followed by mucoepidermoid carcinoma (MEC) (n=37, 18%), squamous cell carcinoma (SCC) (n=19, 9%), adenocarcinoma (n=16, 8%), bronchoalveolar carcinoma (BAC) (n=4, 2%), and small cell carcinoma (SCLC) (n=2, <1%). Factors that significantly affected survival include histology, race, tumor size, lymph node status, and extent of surgery. Patients with MEC and carcinoid tumors had a better overall survival compared to patients with other histologies (p<0.0001). The 5-year overall survival for MEC and carcinoid tumors was 100% and 95% (95% CI 87-98), respectively, versus 50% (95%CI 1-91) for BAC, 28% (95%CI 9-52) for SCC, and 26% (95%CI 5-55) for adenocarcinoma. CONCLUSION The majority of pediatric patients with a primary malignant pulmonary tumor present with carcinoid tumor or MEC and have an excellent prognosis. Lung cancers which are common in adults, but rare in children, have a worse prognosis.

Herlyn-Werner-Wunderlich syndrome: a case report

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital disorder of the Müllerian ducts in which there is uterus didelphys, obstructed hemivagina and unilateral renal agenesis. The most common presentation is an abdominal mass secondary to hematocolpos, pain and dysmenorrhea. However, in some cases, such as the one we present here, menses are normal due to an obstructed hemivagina, and diagnosis can be delayed. We describe evaluation and surgical management of a 13-year-old girl with this condition who was diagnosed by computed tomography (CT) scan and confirmed by pelvic ultrasound and surgical exploration, as well as a review of the literature.

Pediatric papillary thyroid cancer >1 cm: is total thyroidectomy necessary?

PURPOSE Treatment of pediatric papillary thyroid cancer (p-PTC) often follows adult guidelines, including total thyroidectomy for tumors >1cm. This study examined the association between operation type and overall survival (OS) for tumors >1cm in size in the pediatric population. METHODS Patients ≤ 21 years of age with primary papillary thyroid cancer >1cm were reviewed from the National Cancer Data Base (NCDB) from 1998 to 2011. Kaplan-Meier analysis followed by Cox proportional hazard models estimated the impact of total thyroidectomy (TT) vs. partial thyroidectomy (PT) on overall survival. Models were adjusted for patient, tumor, and treatment factors. RESULTS 3,861 cases (3474 TT, 387 PT) were included. Estimated 15-year overall survival was 96.10% after TT and 96.18% after PT (p=0.0855). In multivariate analysis of 3173 patients, only lowest socioeconomic level (HR 4.93, p=0.001) and unfavorable histology (HR 6.11, p=0.016) were associated with worse OS. Survival for patients undergoing TT was not statistically improved over those undergoing PT (HR 0.81, p=0.694). CONCLUSION p-PTC >1cm has an excellent 15-year overall survival. Treatment with TT did not have an improved OS compared to PT. Lower socioeconomic status and unfavorable histology were associated with decreased OS.

Factors associated with mortality in pediatric vs adult nasopharyngeal carcinoma

IMPORTANCE Nasopharyngeal carcinoma (NPC) is endemic in some Asian regions but is uncommon in the United States. Little is known about the racial, demographic, and biological characteristics of the disease in pediatric patients. OBJECTIVES To improve understanding of the differences between pediatric and adult NPC and to determine whether race conferred a survival difference among pediatric patients with NPC. DESIGN, SETTING, AND PARTICIPANTS This retrospective cohort study included all 17 317 patients with a primary diagnosis of NCP in the National Cancer Data Base from January 1, 1998, to December 31, 2011. Of these, 699 patients were 21 years or younger (pediatric); 16 618 patients, older than 21 years (adult). Data were analyzed after data collection. EXPOSURE Pediatric age at diagnosis of NPC. MAIN OUTCOMES AND MEASURES Demographic, tumor, and treatment characteristics of pediatric patients with NPC were compared with those of adults using the χ2 test for categorical variables. An adjusted Cox proportional hazards regression model was used to examine survival differences in pediatric patients relative to adult patients. In addition, the risk for pediatric mortality by race was estimated. RESULTS Of the 17 317 patients, a total of 699 pediatric and 16 618 adult patients were identified with a primary diagnosis of NPC (female, 239 pediatric patients [34.2%] and 5153 adult patients [32.4%]). Pediatric patients were most commonly black (299 of 686 [43.6%]), whereas adults were most likely to be non-Hispanic white (9839 of 16 504 [60.0%]; P < .001). Pediatric patients were less likely to be Asian (39 of 686 [5.7%]) than were adults (3226 of 16 405 [19.7%]; P < .001). Pediatric patients were more likely to have regional nodal evaluation and to present with stage IV disease (227 of 643 [35.3%] and 330 of 565 [58.4%], respectively) than were adult patients (3748 of 15 631 [24.0%] and 6553 of 13 721 [47.8%], respectively; P < .001 for both comparisons). Pediatric patients had a lower risk for mortality relative to adults (hazard ratio, 0.37; 95% CI, 0.25-0.56). No difference in mortality by racial group was found among pediatric patients (hazard ratio, 1.10; 95% CI, 0.82-1.40). CONCLUSIONS AND RELEVANCE Pediatric patients with NPC were more commonly black and presented more frequently with stage IV disease. Pediatric patients had a decreased mortality risk relative to adults, even after adjusting for covariables. Asian race was not associated with increased mortality in pediatric patients with NPC. Racial differences are not associated with an increased risk for mortality among pediatric patients.

Characteristics and outcomes in children with undifferentiated embryonal sarcoma of the liver: A report from the National Cancer Database.

To examine patient characteristics and outcomes in children with undifferentiated embryonal sarcoma of the liver (UESL) using a multi‐institutional database.

The association between nephroblastoma-specific outcomes and high versus low volume treatment centers

BACKGROUND Though the volume-outcome relationship has been well-established in adults, low mortality rates and small sample sizes have precluded definitive demonstration in children. This study compares treatment-specific factors for children with nephroblastoma at high (HVC) versus low volume centers (LVC). METHODS We performed a retrospective cohort study comparing patients ≤18years with unilateral nephroblastoma treated at HVCs and LVCs using the National Cancer Data Base (1998-2012). Definitions of HVCs included performing above the median, the upper two quartiles, and the highest decile of nephroblastoma resections. Outcomes included nodal sampling, margin status, time to chemotherapy and radiation, and survival. Statistical analyses included χ2, t-tests, generalized linear, and Cox regression models (p<0.05). RESULTS Of 2911 patients from 210 centers, 1443 (49.6%) were treated at HVCs. There was no difference in frequency of preoperative biopsy or days to radiation (p>0.05). High volume centers were more likely to perform nodal sampling (RR 1.04, 95%CI 1.01-1.08) and had fewer days to chemotherapy (RR 0.80, 95%CI 0.69-0.93). Five-year survival was similar (HVC: 0.93, 95%CI 0.92-0.94; LVC: 0.93, 95%CI 0.91-0.94). CONCLUSIONS HVCs were more likely to perform nodal sampling and had fewer days to chemotherapy. There was no difference in days to radiation or survival between centers. LEVEL OF EVIDENCE Level II (retrospective prognosis study).

Survival and Surgical Outcomes for Pediatric Head and Neck Melanoma

Importance Melanoma in children is rare, accounting for approximately 2% of all pediatric malignant neoplasms. However, for the past 30 years, the incidence of melanoma in those younger than 20 years has been increasing. Location of the primary tumor has been shown to be an important prognostic factor, with melanomas of the scalp and neck conferring a worse prognosis than those originating at other sites. Objective To examine the survival, demographic, tumor, and treatment characteristics of pediatric head and neck melanoma. Design, Setting, and Participants We performed a retrospective cohort study using information from the National Cancer Data Base from January 1, 1998, to December 31, 2012, on pediatric (⩽18 years) and adult (>18 years) patients with head and neck melanoma. Data analysis was conducted from August 1, 2015, to June 30, 2016. Exposure Pediatric age (⩽18 years) at diagnosis of head and neck melanoma. Main Outcomes and Measures Survival differences were estimated using a Cox proportional hazards regression model. Surgical outcomes, including nodal sampling and margin status, were estimated with generalized linear models comparing pediatric and adult patients. Patient demographic, tumor, and treatment characteristics were estimated using t tests and &khgr;2 tests between pediatric and adult patients with head and neck melanoma for continuous and categorical data, respectively. Results Of the 84 744 patients with head and neck melanoma, 657 (0.8%) were 18 years or younger (mean [SD] age, 13.5 [4.7] years; 285 female and 372 male; 610 white). Pediatric and adult patients had similar demographics but different histologic subtypes (risk difference of pediatric vs adult patients: melanoma, not otherwise specified, 8.5% [95% CI, 4.7%-12.3%]; superficial spreading, 4.2% [95% CI, 0.89%-7.4%]; and lentigo maligna, –13.4% [95% CI, –14.1% to 12.6%]). Pediatric patients had tumors of similar mean depth to those in adult patients (pediatric, 1.54 mm; adult; 1.39 mm; absolute difference, 0.15 mm; [95% CI, –0.32 to 0.008]) and more frequent nodal metastases than did adult patients (risk difference of pediatric vs adult patients for stage T2, 23.9% [95% CI, 14.1%-33.6%]). Five-year survival among pediatric patients was higher for those with stage 1, 2, or 3 disease (absolute difference of pediatric vs adult patients: stage 1, 18% [95% CI, 9.7%-26.3%]; stage 2, 36% [95% CI, 25.3%-46.7%]; stage 3, 39% [95% CI, 26.8%-51.2%]; and stage 4, 2% [95% CI, –8.2% to 12.2%]). Conclusions and Relevance Although pediatric patients with head and neck melanoma present with similar tumor depth and more frequent nodal metastases than do adult patients, younger patients have higher overall survival.

Testicular tumors in prepubescent patients

BACKGROUND/PURPOSE Pediatric testicular tumors are rare, constituting only 1% of all pediatric solid tumors. Single-institution studies addressing pediatric testicular tumors published to date have been limited in the number of patients. METHODS We utilized the National Cancer Data Base (1998-2012) to review all prepubescent patients (≤12 years old) with testicular neoplasms. Demographics, tumor characteristics, treatment modalities, and outcomes were abstracted. RESULTS A total of 479 patients were identified, with a median age of 3 years (IQR 0-4) at diagnosis. 67% of cases were diagnosed by 3 years of age. Yolk sac tumors were the most common histology (202 patients, 42.2%). Most tumors were diagnosed at a low stage. Resection was performed in 465 boys, with 75% having undergone radical orchiectomies. Chemotherapy was utilized in 28% of cases and radiotherapy in 7%. With mean follow-up of 5.6 years, mortality rate was 3%. No difference in mortality was noted based on histology or extent of surgical resection. CONCLUSIONS This series of prepubertal testicular tumors is the largest yet reported and highlights the patient demographics, tumor characteristics, treatment modalities and outcomes for these tumors. TYPE OF STUDY Prognosis study LEVEL OF EVIDENCE: II.