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42. Evaluating Skeletal Maturity at Time of Surgical Correction of Pectus Excavatum Based on Medial Clavicle Epiphyseal Ossification

Author

Carter, Michela, Prendergast, Francis, Krauss, Jillian, Zeineddin, Suhail, Pitt, J. Benjamin, Sullivan, Gwyneth A., Abdullah, Fizan, Gulack, Brian C., and Goldstein, Seth D.

Abstract

Background Surgical correction of pectus excavatum (SCOPE) is dependent upon chest wall pliability with optimal timing prior to complete skeletal maturation. Measures of skeletal maturity are not readily available for operative planning; therefore, surgeons use age as proxy despite patient-specific rates of skeletal maturation. We aimed to determine whether preoperative skeletal maturity is associated with postoperative pain as surrogate for chest wall pliability.Methods Children ≤18 years who underwent SCOPE from 2020 to 2022 were retrospectively identified. Preoperative CT within 3 months of procedure was reviewed by 2 radiologists and 1 surgeon. Skeletal maturity was determined by Schmeling-Kellinghaus classification which stages secondary epiphyseal ossification of the medial clavicle. Inter-rater reliability was evaluated. Schmeling-Kellinghaus stage and postoperative pain were compared.Results Of twenty-eight records reviewed, 57% were Schmeling-Kellinghaus stage 1. High inter-rater reliability was identified (inter-radiologist: kappa = .95, P< .001, all raters: kappa = .78, P< .001). Median age at operation was 15.5 years (interquartile range: 14.8-16.0) and increased with skeletal maturity (P< .001). When comparing stage 1 (n = 16) to >1 (n = 12), stage 1 had lower maximum pain scores (P< .001), total morphine equivalents (P< .001), and benzodiazepine use (P< .001) after surgery.Conclusions The Schmeling-Kellinghaus classification system is a valid proxy of skeletal maturity that can be applied with high inter-rater reliability. SCOPE during stage 1 was found to have less postoperative pain and narcotic use than more mature stages. This is proof of concept that skeletal maturity should be considered when determining optimal timing of surgical correction. Future research will evaluate the impact of skeletal maturity on postoperative outcomes.

Published
2024
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44. Agminated presentation of fusion‐driven melanocytic neoplasms.

Author

Fumero‐Velázquez, Mónica, Hagstrom, Michael, Dhillon, Soneet, Olivares, Shantel, Jennings, Lawrence J., Dittman, David, Sukhanova, Madina, Arva, Nicoleta C., Goldstein, Seth D., Theos, Amy, Pavlidakey, Peter, Carr, Zachary, and Gerami, Pedram

Subjects
FLUORESCENCE in situ hybridization, MELANOMA, TUMORS, CANCER invasiveness
Abstract

Background: The conventionally understood pathogenesis of agminated Spitz nevi includes a mosaic HRAS mutation followed by copy number gains in 11p. However, we have recently observed agminated presentations of fusion‐driven melanocytic neoplasms. Methods: We retrieved cases from our database of benign fusion‐induced melanocytic neoplasms with an agminated presentation. Both the primary lesion and the secondary lesion were sequenced. TERT‐promoter mutational testing and the melanoma fluorescence in situ hybridization assay were also performed. Results: Three cases were included. Two had a PRKCA fusion (partners ATP2B4 and MPZL1) and one had a ZCCHC8::ROS1 fusion. None of the cases met morphologic or molecular criteria for malignancy. There was no evidence of tumor progression in secondary lesions. The same fusion was identified in the primary and secondary lesions. None of the patients developed evidence of nodal or systemic metastasis. Conclusions: We present accumulating evidence that fusion‐driven melanocytic neoplasms can present with an agminated presentation. The differential diagnosis of an agminated presentation versus a locally recurrent or potentially locally metastatic tumor is critical, and accurate diagnosis has significant prognostic and therapeutic consequences for the patient. As with HRAS mutations, fusion‐driven melanocytic tumors may have an agminated presentation. [ABSTRACT FROM AUTHOR]

Published
2023
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