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CD68 immunohistochemical stain shows patchy cytoplasmic expression (C, ×400) gl DISCUSSION LeBoit et al first described this tumor in 1991 using the term "primitive polypoid granular cell tumor".[2] Since then, it has been diagnosed in non-polypoid lesions; therefore, the term "non-neural granular cell tumor" has been proposed. 2005; 29 (7): 927 - 934. doi: 10.1097/01.pas.0000157294.55796.d3 4 Chaudhry IH, Calonje E. Dermal non-neural granular cell tumour (so-called primitive polypoid granular cell tumour): a distinctive entity further delineated in a clinicopathological study of 11 cases. Keywords: anaplastic lymphoma kinase; cutaneous neoplasm; dermatopathology; non-neural granular cell tumor; rapidly growing EN anaplastic lymphoma kinase cutaneous neoplasm dermatopathology non-neural granular cell tumor rapidly growing 1040 1043 4 12/01/22 20221201 NES 221201 INTRODUCTION Non-neural granular cell tumor (NNGCT; also known as primitive polypoid GCT) is a rare neoplasm first described by LeBoit et al in 1991 and is considered a diagnosis of exclusion.[[1]] In contrast to classic GCTs, NNGCTs are immunohistochemically distinct, lacking expression of markers of neural differentiation, such as SOX10 and S-100. [Extracted from the article]

Background: The Children's Oncology Group (COG) adopted cisplatin, 5‐flourouracil, and vincristine (C5V) as standard therapy after the INT‐0098 legacy study showed statistically equivalent survival but less toxicity in comparison with cisplatin and doxorubicin. Subsequent experience demonstrated doxorubicin to be effective in patients with recurrent disease after C5V, and this suggested that it could be incorporated to intensify therapy for patients with advanced disease. Methods: In this nonrandomized, phase 3 COG trial, the primary aim was to explore the feasibility and toxicity of a novel therapeutic cisplatin, 5‐flourouracil, vincristine, and doxorubicin (C5VD) regimen with the addition of doxorubicin to C5V for patients considered to be at intermediate risk. Patients were eligible if they had unresectable, nonmetastatic disease. Patients with a complete resection at diagnosis and local pathologic evidence of small cell undifferentiated histology were also eligible for an assessment of feasibility. Results: One hundred two evaluable patients enrolled between September 14, 2009, and March 12, 2012. Delivery of C5VD was feasible and tolerable: the mean percentages of the target doses delivered were 96% (95% CI, 94%‐97%) for cisplatin, 96% (95% CI, 94%‐97%) for 5‐fluorouracil, 95% (95% CI, 93%‐97%) for doxorubicin, and 90% (95% CI, 87%‐93%) for vincristine. Toxicity was within expectations, with death as a first event in 1 patient. The most common adverse events were febrile neutropenia (n = 55 [54%]), infection (n = 48 [47%]), mucositis (n = 31 [30%]), hypokalemia (n = 39 [38%]), and elevated aspartate aminotransferase (n = 28 [27%]). The 5‐year event‐free and overall survival rates for the 93 patients who did not have complete resection at diagnosis were 88% (95% CI, 79%‐93%) and 95% (95% CI, 87%‐98%), respectively. Conclusions: The addition of doxorubicin to the previous standard regimen of C5V is feasible, tolerable, and efficacious, and this suggests that C5VD is a good regimen for future clinical trials. The addition of doxorubicin to the previous standard regimen of cisplatin, 5‐flourouracil, and vincristine is a feasible, tolerable, and efficacious novel treatment regimen (cisplatin, 5‐flourouracil, vincristine, and doxorubicin). This regimen is a reasonable option for future clinical trials, especially those including patients with unresectable disease at diagnosis. [ABSTRACT FROM AUTHOR]

Background: The identification of new therapies for high-risk (HR) hepatoblastoma is challenging. Children's Oncology Group study AHEP0731 included a HR stratum to explore the efficacy of novel agents. Herein, the authors report the response rate to the combination of vincristine (V) and irinotecan (I) and the outcome of patients with high-risk hepatoblastoma.Methods: Patients with newly diagnosed metastatic hepatoblastoma or those with a serum α-fetoprotein (AFP) level <100 ng/mL were eligible. Patients received 2 cycles of V at a dose of 1.5 mg/m2 /day intravenously on days 1 and 8 and I at a dose of 50 mg/m2 /day intravenously on days 1 to 5. Patients were defined as responders if they had either a 30% decrease in tumor burden according to Response Evaluation Criteria In Solid Tumors (RECIST) or a 90% (>1 log10 ) decline in their AFP level. Responders were to receive 2 additional cycles of VI intermixed with 6 cycles of the combination of cisplatin, doxorubicin, 5-fluorouracil, and vincristine (C5VD). Nonresponders were to receive 6 cycles of C5VD alone.Results: A total of 32 patients with a median age at diagnosis of 26 months (range, 11-159 months) were enrolled between September 2009 and February 2012. Fourteen of 30 evaluable patients were responders (RECIST and AFP in 6 patients, RECIST only in 3 patients, and AFP only in 5 patients). The median AFP decline after 2 cycles of VI for the entire group was 345,565 ng/mL (85% of the initial AFP). The 3-year event-free and overall survival rates were 49% (95% confidence interval, 30%-65%) and 62% (95% confidence interval, 42%-77%), respectively.Conclusions: The VI combination appears to have substantial activity against HR hepatoblastoma. The ultimate impact of this regimen in improving the outcomes of children with HR hepatoblastoma remains to be determined. Cancer 2017;123:2360-2367. © 2017 American Cancer Society. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented in response to the article "''Successfulnontransplant resection of POST-TEXT III and IV hepatoblastoma" by Lautz et al in the 2012 issue.