Introduction: Melanotic neuroectodermal tumour of infancy (MNTI) is a benign tumour of infancy, most commonly affecting the head and neck region. First described in 1918, less than 500 cases have been reported in the literature. MNTI is aggressive in nature & has a high rate of recurrence.
Presentation Of Cases: In this retrospective case series, we report two cases of MNTI that presented at our unit; both cases were managed by wide excision and have been followed up uneventfully for over two years.
Discussion: MNTI has a recurrence rate of up to 20%. Patient's age can play a significant role in recurrence rate. Although this neural crest tumour is somewhat rare in the literature, there is a consensus with regards to surgical management; the gold standard remains to be wide excision with safety margin. Select cases may benefit from adjuvant and neoadjuvant therapy.
Conclusion: Owing to its locally aggressive nature and high recurrence rate, prompt diagnosis and surgical intervention is advised in cases of MNTI. Further understanding of this tumour is needed on a microscopic level in order to determine clear prognostic factors.
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http://dx.doi.org/10.1016/j.ijscr.2018.11.004 | DOI Listing |
Orphanet J Rare Dis
January 2025
Division of Pediatric Epileptology, Department of Pediatrics I, Medical Faculty of Heidelberg, Heidelberg University, Heidelberg, Germany.
Background: Tuberous sclerosis complex (TSC) is an autosomal dominant genetic disorder affecting multiple organ systems, with a prevalence of 1:6,760-1:13,520 live births in Germany. On the molecular level, TSC is caused by heterozygous loss-of-function variants in either of the genes TSC1 or TSC2, encoding the Tuberin-Hamartin complex, which acts as a critical upstream suppressor of the mammalian target of rapamycin (mTOR), a key signaling pathway controlling cellular growth and metabolism. Despite the therapeutic success of mTOR inhibition in treating TSC-associated manifestations, studies with mTOR inhibitors in children with TSC above two years of age have failed to demonstrate beneficial effects on disease-related neuropsychological deficits.
View Article and Find Full Text PDFZhonghua Bing Li Xue Za Zhi
January 2025
Department of Pathology, Children's Hospital of Fudan University Anhui Hospital (Anhui Provincial Children's Hospital), Hefei230051, China.
Crit Rev Oncol Hematol
December 2024
Department of Urology, Institute of Urology, West China Hospital, Sichuan University, Chengdu 610041, China. Electronic address:
In recent years, cancer immunotherapy has received widespread attention due to significant tumor clearance in some malignancies. Various immunotherapy approaches, including vaccines, immune checkpoint inhibitors, oncolytic virotherapy, bispecific T cell engagers, and adoptive T cell transfer, have completed or are undergoing clinical trials for prostate cancer. Despite immune checkpoint blockade's extraordinary effectiveness in treating a variety of cancers, targeted prostate cancer treatment using the immune system is still in its infancy.
View Article and Find Full Text PDFInt J Surg Case Rep
December 2024
Department of Radiology, B and B Hospital, Gwarko, Lalitpur, Nepal.
Introduction And Importance: Growing skull fracture is one the rarest complication of skull fracture. They are identified mostly in infancy and children's, secondary to head trauma but there exist possibility of missed diagnosis in childhood and patient presenting in adulthood with delayed complications. Hence timely diagnosis and prompt management is mandatory.
View Article and Find Full Text PDFPediatr Dermatol
December 2024
Section of Dermatology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.
Infantile hemangiomas (IH) are the most common benign tumors of infancy and progress through recognized stages of evolution including early proliferation, plateau, and involution. Ulceration is a common complication of IHs typically observed during the early proliferative stage characterized by rapid growth. In rare cases, ulceration is the primary clinical manifestation of IHs.
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