Daniela Massi1, Carlo Tomasini2, Rebecca Senetta3, Milena Paglierani4, Francesca Salvianti5, Maria Elena Errico6, Vittoria Donofrio6, Paola Collini7, Gabrina Tragni8, Angela Rita Sementa9, Franco Rongioletti10, Renata Boldrini11, Andrea Ferrari12, Claudio Gambini9, Maria Cristina Montesco13. 1. Department of Surgery and Translational Medicine, University of Florence, Florence, Italy. Electronic address: daniela.massi@unifi.it. 2. Dermatopathology Section, Azienda Ospedaliera Città della Salute e della Scienza, Turin, Italy. 3. Department of Medical Sciences, University of Turin, Turin, Italy. 4. Department of Surgery and Translational Medicine, University of Florence, Florence, Italy. 5. Department of Biomedical, Experimental, and Clinical Sciences, University of Florence, Florence, Italy. 6. Pediatric Hospital Santobono-Pausilipon, Naples, Italy. 7. Unit of Soft Tissue and Bone Pathology, Histopathology, and Pediatric Pathology, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Istituto Nazionale dei Tumori, Milan, Italy. 8. Unit of Dermatopathology, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Istituto Nazionale dei Tumori, Milan, Italy. 9. Istituto Giannina Gaslini, Genoa, Italy. 10. IRCSS AOU S. Martino, Department of Health Sciences, DISSAL, University of Genoa, Genoa, Italy. 11. Bambino Gesù Children's Hospital, Rome, Italy. 12. Unit of Pediatric Oncology, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Istituto Nazionale dei Tumori, Milan, Italy. 13. Veneto Institute of Oncology Istituto Oncologico Veneto Istituto di Ricovero e Cura a Carattere Scientifico (IOV IRCCS), Padua, Italy.
Abstract
BACKGROUND: Diagnosis and proper management of atypical Spitz tumors in pediatric age are still controversial. OBJECTIVE: We sought to investigate the clinicopathological and molecular features of atypical Spitz tumors in patients aged 18 years or younger. METHODS: We performed a retrospective clinicopathological and fluorescence in situ hybridization study on 50 pediatric atypical Spitz tumors. RESULTS: Parameters that were significantly correlated with a diagnosis of atypical Spitz tumors over Spitz nevus included asymmetry, level IV/V, lack of maturation, solid growth, nuclear pleomorphism, high nuclear-cytoplasmic ratio, atypical and deep mitoses, and more than 6 mitoses/mm(2). In the atypical Spitz tumors group, a significantly higher mitotic rate was observed in prepuberal age (P = .04). The 4-probe fluorescence in situ hybridization melanoma assay did not discriminate atypical Spitz tumors from Spitz nevi. Heterozygous 9p21 loss was found in 3 of 37 cases and homozygous 9p21 loss in 2 of 37 cases. Only 1 child experienced a fatal outcome, showing genetic abnormalities by melanoma fluorescence in situ hybridization probe and a heterozygous 9p21 deletion. LIMITATIONS: The limited number of adverse outcomes did not allow the prognostic analysis of single morphologic features. CONCLUSION: Pediatric atypical Spitz tumors are associated with minimal lethal potential. Atypical Spitz tumors require complete excision and careful follow-up while our data do not support any clinical benefit for the sentinel lymph node biopsy procedure and completion lymphadenectomy.
BACKGROUND: Diagnosis and proper management of atypical Spitz tumors in pediatric age are still controversial. OBJECTIVE: We sought to investigate the clinicopathological and molecular features of atypical Spitz tumors in patients aged 18 years or younger. METHODS: We performed a retrospective clinicopathological and fluorescence in situ hybridization study on 50 pediatric atypical Spitz tumors. RESULTS: Parameters that were significantly correlated with a diagnosis of atypical Spitz tumors over Spitz nevus included asymmetry, level IV/V, lack of maturation, solid growth, nuclear pleomorphism, high nuclear-cytoplasmic ratio, atypical and deep mitoses, and more than 6 mitoses/mm(2). In the atypical Spitz tumors group, a significantly higher mitotic rate was observed in prepuberal age (P = .04). The 4-probe fluorescence in situ hybridization melanoma assay did not discriminate atypical Spitz tumors from Spitz nevi. Heterozygous 9p21 loss was found in 3 of 37 cases and homozygous 9p21 loss in 2 of 37 cases. Only 1 child experienced a fatal outcome, showing genetic abnormalities by melanoma fluorescence in situ hybridization probe and a heterozygous 9p21 deletion. LIMITATIONS: The limited number of adverse outcomes did not allow the prognostic analysis of single morphologic features. CONCLUSION: Pediatric atypical Spitz tumors are associated with minimal lethal potential. Atypical Spitz tumors require complete excision and careful follow-up while our data do not support any clinical benefit for the sentinel lymph node biopsy procedure and completion lymphadenectomy.
Authors: D W Bartenstein; J M Fisher; C Stamoulis; C Weldon; J T Huang; S E Gellis; M G Liang; B Schmidt; E B Hawryluk Journal: Br J Dermatol Date: 2019-02-10 Impact factor: 9.302
Authors: Thomas Wiesner; Heinz Kutzner; Lorenzo Cerroni; Martin C Mihm; Klaus J Busam; Rajmohan Murali Journal: Pathology Date: 2016-01-18 Impact factor: 5.306