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Simple Summary Melanotic Neuroectodermal Tumor of Infancy (MNTI) is a very rare neoplasm that most commonly develops within maxilla in infants. It usually has a benign clinical course and is treated with only surgery. However, patients with large, inoperable, metastatic or multiply recurring MNTI may require systemic treatment. The role of pre- and post-surgery chemotherapy (CHT) in the management of MNTI is unclear. Here, we have presented the disease courses and outcomes of four infants treated with multidrug CHT due to inoperable/recurrent MNTI. Additionally, a systematic literature review was performed which revealed 38 similar cases in the last 42 years. Most children with primarily inoperable MNTI responded to CHT, which allowed physicians to perform complete, non-mutilating delayed surgery. However, it is still uncertain whether CHT administered after incomplete resection of MNTI prevents recurrence. This study aimed to contribute to the establishment of standards of management in patients with inoperable, metastatic or persistently recurring MNTIs, which are currently lacking. Abstract Melanotic Neuroectodermal Tumor of Infancy (MNTI) is a very rare pediatric neoplasm of neural crest origin. In most cases, it develops in infants as a localized tumor of the maxilla, and surgery is usually curative. In less than 10% of patients with inoperable, metastatic or persistently recurring MNTI, chemotherapy (CHT) may be considered; however, its role is still unclear. The aim of our study was to assess the efficacy of CHT in children with large, inoperable, metastatic and/or recurrent MNTI. Four such infants, treated with CHT in Polish and German centers of pediatric oncology, were presented. Additionally, a systematic literature search of the PubMed/MEDLINE, Scopus and Web of Science databases was performed, yielding 38 similar cases within the last 42 years. Neoadjuvant CHT, based mainly on the protocols for neuroblastoma, was often effective, allowing for complete delayed surgery in most cases. However, the role of adjuvant CHT in preventing recurrences after incomplete resection of MNTI remains unclear. Disseminated inoperable MNTI was almost universally associated with poor response to CHT and unfavorable outcome. Further investigations to elaborate standards of management in patients with inoperable, metastatic or persistently recurring MNTIs are necessary to improve outcomes.

Perineural infiltration (PNI) and desmoplasia are believed to be high-risk factors in the prognosis of squamous cell carcinoma (SCC). In the literature, dependences between PNI, de-differentiation, and desmoplasia remain unclear. The aim of this study was to analyze the respective prognostic impact of these factors in regard to local recurrence and metastasis. Between 2005 and 2015, 1,399 unselected primary SCCs of 1,434 patients were diagnosed. If a patient had multiple tumors, the tumor with the highest risk profile was selected. Histological sections of all tumors with a tumor thickness of ≥6 mm and desmoplastic SCC with a tumor thickness of 2.1–5.9 mm were re-examined for PNI. Median follow-up was 36.5 months. PNI was present exclusively within tumors of the desmoplastic type (14.5%). PNI was present significantly more often in patients developing lymph node metastasis (3% all non–desmoplastic SCC, 17% desmoplastic SCC, and 29% desmoplastic SCC with PNI) and local recurrence (3%, 26%, and 64%) and associated with overall tumor-specific death (4%, 25%, and 54%). Using a multivariate model of disease recurrence, tumor thickness ≥6 mm, tumor horizontal size ≥20 mm, immunosuppression, desmoplasia, and PNI remained significant factors. In conclusion, PNI was found to be an additional marker indicative of an unfavorable prognosis and an independent high-risk factor within the desmoplastic type of SCC.

Background: Perusal of the literature of cutaneous squamous cell carcinoma reveals that the role of the desmoplastic type is indistinct. The aim of this prospective study was to analyse local infiltration, recurrence and resulting outcome of the desmoplastic type. Methods: Between 2005 and 2015 2149 unselected primary cutaneous squamous cell carcinomas of 1434 patients were diagnosed and treated at the Department of Dermatology, University of Tuebingen/Germany. Data collection was based on prospectively collected data during follow-up of median 36.5 months for tumor infiltration and loco-regional recurrence rate, as well as tumor-specific death, rate of metastasis and perineural infiltration. From this cohort a sample of 320 tumors (non-desmoplastic type, n= 260; desmoplastic type, n= 60) were randomly selected to investigate surgical and histological details. Findings: Desmoplastic type required more re-excisions after initial resection (70.0% vs. non-desmoplastic type 23.9%, p