Long-term response with adjuvant chemotherapy for recurrent Melanocytic Neuroectodermal Tumor of Infancy

Long-term response with adjuvant chemotherapy for recurrent Melanocytic Neuroectodermal Tumor of Infancy

Sophia Taylor *¹, Aminu Abdurrhman ¹, Harman Narang ¹, Obyda Mahrous ¹, Zahra Gill ¹,
Hamza Mahrous ¹, Haris Taylor 1, Othman Mahrous ¹, Ihsan Adeleke ¹

1. MEDPASS Fellowship Students, Dallas, Texas.

*Correspondence to: Sophia Taylor, MEDPASS Fellowship Students, Dallas, Texas.

Copyright
© 2026 Sophia Taylor is an open access article distributed under the Creative Commons Attribution   License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Received: 04 October 2026

Published: 01 November 2026

DOI: https://doi.org/10.5281/zenodo.23907166

Abstract

Melanocytic neuroectodermal tumor of infancy (MNTI) is a rare, pigmented tumor generally occurring in the head and neck region in children 12 months of age or younger. As of 2016, only 472 cases have been reported in the literature. Although usually a benign tumor, MNTI can demonstrate locally invasive behavior and requires surgical resection as a first-line treatment.

Only around 4% of all reported cases in the literature have included chemotherapy at any stage in the treatment of MNTI. This has been primarily limited to its use as an adjuvant in patients with metastases or recurrent disease, often with limited benefit. We report here a child who remains disease-free at the 7-year follow-up, having been successfully treated using an OPEC/OJEC regimen for recurrent MNTI following incomplete surgical resection. We present a review of the literature and discuss the surgical and chemotherapeutic complications that arose in the management of this case to help inform future management of patients.

Introduction

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare, fast-growing neoplasm of neural crest origin, usually arising in the craniofacial bones of an infant. The first report of MNTI was in 1918, where Krompecher first described a ‘congenital melanocarcinoma’ (1). There have since been twenty three synonyms for MNTI reported in the literature, including melanotic prognoma, melanotic adamantinoma, pigmented epulis and retinal anlage tumor (2). In 1966, Borello and Gorlin described a subset of MNTI patients with an elevated urinary concentration of vanillylmandelic acid (VMA), pointing to a shared origin with other neuroectodermal tumors such as neuroblastoma and phaeochromocytoma (3). Corroborating reports described an increased urinary VMA concentration that returned to normal following excision of the tumor (4, 5). Since then, ultrastructural, immunohistochemical and tissue culture studies have supported the neural crest origin of these tumors (6-8).

Clinical Presentation

A systematic review by Rachidi et al. (9) found 472 reported cases in the literature from 1918 to 2013. MNTI primarily presents during the first year of life (10) with a slight male predominance (1.3:1, male: female). The median age at diagnosis was 4.5 months (IQR, 3 months), comparable between both sexes (9). The commonest sites of origin are the maxilla (62.2%), skull (15.6%), and mandible (7.8%) (9). Other cases have been reported in the epididymis (11), ovary (12), uterus (13), mediastinum (14) and peripheral bones (15).

Histopathology

Microscopically, these tumors are composed of two different cell types arranged in irregular nest-like formations separated by dense, well-vascularized, collagenous stroma. The first cell type consists of small, round-shaped cells with scanty cytoplasm, hyperchromatic nuclei and dense vesicles observed under electron microscopy (7). These neuroblastoma-like cells may be identified immunohistochemically by neuron-specific enolase and synaptophysin staining (16, 17). The second cell type consists of melanin-containing cuboidal epithelium arranged in alveolar or tubular structures surrounding clusters of the small round cells. The Fontana-Masson silver stain elucidates the melanin production in these larger cells, which may further be identified by cytokeratin and HMB-45 expression (7, 8). Although not exclusively present, these larger pigmented cells can help to distinguish MNTI from a list of differential diagnoses for the pediatric ‘small round cell’ neoplasms, including neuroblastoma, primitive neuroectodermal tumor, rhabdomyosarcoma, Ewing’s sarcoma and lymphoma (18). Other markers which have been found to be positively staining in MNTI samples include vimentin, chromogranin A and CD56, however Melan A and glial fibrillar acidic protein are usually not expressed (18-20). One case series of eight patients described a correlation between Ki-67 and CD99 expression and tumor aggressiveness, however there is limited evidence to support the use of histological or immunohistochemical markers in prognosis (19).

Clinical Behavior & Management

Although usually a benign tumor, MNTI can demonstrate locally invasive behavior which may be fatal if vital tissues are involved (21). Rarely, the tumor can develop malignant potential with an estimated rate of metastasis of 3% (22). Cases of metastasis have been reported in regional lymph nodes, brain, liver, pelvis, orbit and bone marrow (7, 17, 22-24).

The mainstay of treatment for MNTI is surgery, which is curative in most cases. However, the local recurrence rate at 5 years from initial diagnosis is 23% (9), which could reflect either cases of incomplete resection, intraoperative dissemination or multicentricity (25). There still exists controversy around the extent of marginal clearance in surgical resection (26). A dilemma exists to allow residual portions of tumor to remain if radical excision would be mutilating, with the option of further treatment by adjuvant chemotherapy still available (4, 27). However, other reports have recommended more aggressive treatment where possible, with wide excision of the primary tumor including a layer of normal bone around the margins (28). Several reports have adhered to a recommended resection margin of 5mm (19, 29-31).

Chemotherapy

The use of chemotherapy in MNTI has yielded conflicting reports on effectiveness, in general showing limited responses (32-34). For example, Dehner et al. (7) reported a case of metastatic maxillary MNTI which progressed whilst on treatment with cyclophosphamide and high dose methotrexate with vincristine. In contrast, Woessmann et al. (10) reported a complete response to an aggressive “8 in 1” chemotherapeutic regimen consisting of vinblastine, ifosfamide/cyclophosphamide, etoposide, doxorubicin/liposomal daunorubicin and dactinomycin, observed in a 2 month old infant with locally aggressive maxillary MNTI. The child avoided mutilating surgery and was free of tumor recurrence at the age of two years. However, advocacy for the use of chemotherapy has largely been limited to the contexts of recurrent or metastatic disease and as an adjuvant in cases where complete surgical resection cannot be achieved (35, 36).  A number of case reports documenting the use of radiotherapy in the management of MNTI have not shown any benefit from this method of treatment (5).

In one report, partial remission of a metastatic frontal tumor was observed with etoposide and cisplatin and a good partial response was obtained in a metastatic MNTI of the femur using an alternating chemotherapy regime of cyclophosphamide/doxorubicin with cisplatin/etoposide/vincristine (37). Chemotherapy has also been used in a neoadjuvant context, successfully enabling surgery on a 6 month old girl presenting with a large maxillary MNTI which was initially inoperable because of the extent of local invasion (35).

Detailed Case

Presentation

A 5 week-old boy presented with a rapidly growing lump behind his right ear which his mother had first noted on day 9 after birth. He was born at term and there were no complications in pregnancy or in the early neonatal period. There was no family history of malignancy.On examination there was a large 3 cm mass pushing the right pinna forward which was firm and non-tender. There was no neck lymphadenopathy. He was otherwise a contented baby who was systemically well with an unremarkable general examination.

Computed tomography (CT) with contrast showed a soft tissue mass (2.1 x 1.5 cm) posterior to the right ear with no calcification present (Figure 1).  There appeared to be mild compression of the skull vault, no communication or intracranial lesion.  Routine hematology and biochemistry including full blood count, liver function tests, bone profile and electrolytes were all normal.  Urinary VMA/creatinine ratio and HVA/creatinine ratio were both normal.  Staging investigations including MRI of the whole neuroaxis, chest and abdomen CT scan, bone scan and bone marrow aspirate and trephine showed no evidence of distant metastasis.

Initial Surgery

At 6 weeks old, our patient underwent an operation to resect the tumor and for definitive diagnosis, a biopsy was sent for histology. Light microscopy revealed sheets of connective tissue with diffuse infiltration of small round blue cells with ‘salt and pepper’ chromatin and brown pigment deposits within them. Immunohistochemistry showed positive staining for synaptophysin, NB-84, CD56 and BAF-47, confirming the diagnosis of MNTI. However, histology showed tumor cells at the margin of resection and a post-operative MRI showed some localized thickening of the skull in the supra-auricular area at the site of the tumor. The resection was presumed incomplete with microscopic residual disease. After discussion at our multidisciplinary team meeting, we decided not to opt for further excision or chemotherapy at that stage but to closely monitor the patient for local recurrence or metastatic disease.

Recurrence: Chemotherapy and Further Surgery

Unfortunately, four months later, his mother noticed a recurrence of the lump behind his right ear but the infant was otherwise well. MRI showed a mass related to the inner table of the right posterior temporal region measuring 17 x 27mm in the axial plane with significant enhancement. Compared to the post-operative MR images, this showed clear progression which was thought to be dural based, possibly arising from bone. Spinal MRI showed subtle expansion and enhancement in the distal cord and conus suggesting metastatic spread although this was not clearly substantiated on axial images and CSF examination was negative.

With the recurrence of disease and suggestion of metastatic spread in the cord, surgery was not considered to be an appropriate first option and so chemotherapy was started. On 19 February 2009, he was commenced on OPEC/OJEC chemotherapy (Vincristine, Cisplatin and Etoposide and Cyclophosphamide alternating with Vincristine Carboplatin, Etoposide and Cyclophosphamide; See Table 1 for doses) according to the Children's Cancer & Leukemia Group (CCLG) guidelines for metastatic/unresectable MNTI. After two courses, response was assessed by MRI which showed significantly reduced enhancement of the tumor and a cessation in tumor enlargement.

Table 1: Dose regimens for OPEC/OJEC chemotherapy

He therefore received two further cycles of OPEC/OJEC before undergoing a right temporal tumor excision in April 2009. A wide cortical mastoidectomy was performed removing bone down to the tegman plate and posterior fossa dura. Bone was then removed from the lateral and transverse and venous sinus. The tumor was firmly adherent to the dura and underlying brain parenchyma and had also invaded the sigmoid sinus at the site where the mastoid emissary vein enters. At the conclusion of the procedure, all visible tumor was removed.

The post-operative MRI showed no evidence of residual tumor, however, tissue taken from the area of the sigmoid sinus bone, dura and the edge of sinus were all infiltrated with tumor. Given the good response to pre-operative chemotherapy, he was given another 3 courses of OPEC/OJEC chemotherapy. He developed two further complications of surgery- a post-operative false meningocele and mild herniation of brain parenchyma through a right sided craniectomy defect. This required two further operations- a cranioplasty, which later required revisional surgery.

At the end of OPEC/OJEC chemotherapy regimen lasting 6 months in total (4 cycles pre-operatively and 3 cycles post-operatively; See Table 2), subsequent scans showed no residual or recurrent disease. His kidney function and hearing were within normal limits. At 7 years old (six and half years after the second resection), the patient was doing well, developing normally and disease free on follow-up MRI (See Figure 2). He will continue to have yearly MR scans until 10 years of age.

Table 2: Pre-operative and post-operative chemotherapy regimen

O: vincristine, P: cisplatin, E: etoposide, C: cyclophosphamide, J: carboplatin

Discussion

Only around 4% of reported cases since 1918 have included chemotherapy at any stage in the treatment of MNTI. Its use has been primarily as an adjuvant in patients with metastases (9), often with limited benefit (7). However, in one report, partial remission of a metastatic frontal tumor was observed with etoposide and cisplatin and a good partial response was obtained in a metastatic MNTI of the femur using an alternating chemotherapy regime of cyclophosphamide/doxorubicin with cisplatin/etoposide/vincristine (37).

There are similarly mixed reports about the benefit of neoadjuvant chemotherapy. Ahmed et al. (38) reported a 7 month old boy who presented with a mandibular MNTI and was subsequently treated with nine courses of neoadjuvant chemotherapy (ifosfamide, vincristine and actinomycin D, carboplatin, etoposide and doxorubicin) which failed to control tumor expansion. However, neoadjuvant chemotherapy given to a 6 month old girl who presented with an initially inoperable, locally invasive maxillary MNTI, successfully enabled surgery to go ahead (35).

We present a case of MNTI who underwent successful use of chemotherapy for recurrent disease following an incomplete surgical resection. The child presented with recurrent disease four months after the initial operation and received four cycles of the OPEC/OJEC regimen. Although there was no dramatic tumor shrinkage, which has been very rarely reported in the literature (10), the tumor nevertheless showed a good response. MRI demonstrated an arrest in tumor growth and significantly reduced enhancement, enabling a second resective operation to proceed. Resection margin histology demonstrated residual disease for a second time, so the child received three additional post-operative cycles of OPEC/OJEC, which was again successful. The child remains healthy and disease-free on MRI at the 7 year follow-up.

Given the rarity of MNTI cases managed by chemotherapy, we also discuss here complications of our regimen to help inform future management of cases. On day 3 of the first course of OPEC chemotherapy, our patient became very distressed and developed abdominal distension. Although not neutropenic at the time, a working diagnosis of typhlitis was considered. However, abdominal X-ray and surgical review confirmed a paralytic ileus, which was managed by keeping him nil by mouth and parenteral nutrition. On day 5, he developed a partial ptosis and a hoarse voice, which are both features of vincristine toxicity. We initially gave vincristine 0.05mg/kg, equivalent to the standard dose typically given in older children. However, in clinical trials involving infants, there is typically a two-thirds dose reduction (39), thus upon developing toxicity, this was reduced appropriately.

After 3 weeks of supportive care our patient made a full recovery from the vincristine side effects with no permanent residual problems and on subsequent courses, the vincristine dose was modified.

What is striking about the use of chemotherapy in MNTI is the variability in the use of agents and regimens. Murphy et al. (31) reported described a 3 month old boy who presented with a maxillary MNTI and subsequently underwent surgical resection of the tumor. Like our case, the resection was later found to be incomplete after histological analysis. Unlike out case, instead of watching and waiting for recurrent tumor, he was immediately treated with adjuvant chemotherapy with a 10-week course consisting of weekly vincristine until week 6 and then 3 weekly vincristine, and 3 weekly cyclophosphamide therapies. There was no evidence of recurrence at the 3 year post-surgical follow-up. Of note, the regime shared vincristine and cyclophosphamide in common but was treated with only those two agents alone and in a shorter time frame (10 weeks continuously vs 24 weeks).

Radiotherapy considerations

Radiotherapy has a limited but potentially important role in the treatment of melanocytic neuroectodermal tumors (MNTI), particularly when the tumor is aggressive, recurrent, or cannot be completely removed surgically. Because MNTI is rare and most cases occur in infants, surgical excision is generally the primary treatment. Radiotherapy for local control may be considered for residual or recurrent disease, especially when complete surgical removal is not possible. However, its use is approached cautiously because of the potential long-term effects of radiation in young children, including effects on developing tissues and the risk of secondary malignancies. In this case radiotherapy was avoided and hence any long tern risk associated with this avoided.

In another case with a similar disease timeline to ours, a 5 month old boy received etoposide alone after a second microscopically incomplete resection and was alive without disease at 1 year follow-up (40). Cyclophosphamide, carboplatin and etoposide was used in another case (41). In addition to the case report of Woessmann et al. discussed earlier, only one other report describes a complete, long-term response to chemotherapy alone. This regimen consisted of vincristine, cyclophosphamide, etoposide, doxorubicin and dactinomycin resulting in tumor shrinkage (42). After comparing all of these successes, Woessmann et al. (10) speculate etoposide as being a key agent in inducing neuroectodermal tumor apoptosis, which was also a component in our OPEC/OJEC regimen. However, without larger sample sizes, it is difficult to evaluate the significance of this correlation.

Surgery remains the definitive treatment for MNTI and indeed, most cases of this rare tumor are successfully cured by resection alone. This leaves limited experience in managing cases with chemotherapy, but we report here the successful use of an OPEC/OJEC regimen in the treatment of recurrent MNTI following incomplete resection.

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