# Case Report: Anlotinib combined with radiotherapy achieves sustained disease control in advanced malignant melanotic nerve sheath tumor

**Authors:** Huizhan Zhu, Wei Shen, Han Yang, Xiaoyang Han, Weiwei Li, Jinling Xie, Yong Tan, Ping Lu, Yinghua Ji, Yana Zhang

PMC · DOI: 10.3389/fonc.2026.1680441 · 2026-02-03

## TL;DR

A 54-year-old woman with advanced melanotic nerve sheath tumor showed long-term improvement with anlotinib and radiotherapy.

## Contribution

This is the first reported case demonstrating the effectiveness of anlotinib combined with radiotherapy for MMNST.

## Key findings

- The patient achieved over 32 months of progression-free survival with anlotinib and radiotherapy.
- The combination therapy may offer a promising treatment option for advanced MMNST.
- This case provides clinical evidence for managing a rare and aggressive tumor subtype.

## Abstract

Malignant melanotic nerve sheath tumor (MMNST) is a rare and aggressive subtype of malignant peripheral nerve sheath tumor (MPNST) characterized by melanin-producing cells. Due to its rarity, there is currently no standardized therapeutic strategy, posing significant challenges in selecting effective treatment modalities.

We report the first documented case of a 54-year-old female with recurrent MMNST and multiple pulmonary metastases. Following local radiotherapy combined with systemic anlotinib treatment, the patient achieved prolonged progression-free survival (PFS) exceeding 32 months.

This case suggests that anlotinib combined with radiotherapy may be an effective therapeutic option for advanced MMNST, providing valuable clinical evidence for managing this malignancy.

## Linked entities

- **Chemicals:** anlotinib (PubChem CID 25017411)
- **Diseases:** malignant peripheral nerve sheath tumor (MONDO:0004345)

## Full-text entities

- **Genes:** HIF1A (hypoxia inducible factor 1 subunit alpha) [NCBI Gene 3091] {aka HIF-1-alpha, HIF-1A, HIF-1alpha, HIF1, HIF1-ALPHA, MOP1}, PDGFRA (platelet derived growth factor receptor alpha) [NCBI Gene 5156] {aka CD140A, PDGFR-2, PDGFR2}, MLANA (melan-A) [NCBI Gene 2315] {aka MART-1, MART1}, PRKAR1A (protein kinase cAMP-dependent type I regulatory subunit alpha) [NCBI Gene 5573] {aka ACRDYS1, ADOHR, CAR, CNC, CNC1, PKR1}, TTF1 (transcription termination factor 1) [NCBI Gene 7270] {aka TTF-1, TTF-I}, CD8A (CD8 subunit alpha) [NCBI Gene 925] {aka CD8, CD8alpha, IMD116, Leu2, p32}, PDGFRB (platelet derived growth factor receptor beta) [NCBI Gene 5159] {aka CD140B, IBGC4, IMF1, JTK12, KOGS, OPDKD}, KIT (KIT proto-oncogene, receptor tyrosine kinase) [NCBI Gene 3815] {aka C-Kit, CD117, MASTC, PBT, SCFR}, TFRC (transferrin receptor) [NCBI Gene 7037] {aka CD71, IMD46, T9, TFR, TFR1, TR}, MAPK1 (mitogen-activated protein kinase 1) [NCBI Gene 5594] {aka ERK, ERK-2, ERK2, ERT1, MAPK2, NS13}, S100A1 (S100 calcium binding protein A1) [NCBI Gene 6271] {aka S100, S100-alpha, S100A}, EPHA2 (EPH receptor A2) [NCBI Gene 1969] {aka ARCC2, CTPA, CTPP1, CTRCT6, ECK}, VIM (vimentin) [NCBI Gene 7431], SOX10 (SRY-box transcription factor 10) [NCBI Gene 6663] {aka DOM, PCWH, SOX-10, WS2E, WS4, WS4C}, KDR (kinase insert domain receptor) [NCBI Gene 3791] {aka CD309, FLK1, VEGFR, VEGFR2}, AKT1 (AKT serine/threonine kinase 1) [NCBI Gene 207] {aka AKT, PKB, PKB-ALPHA, PRKBA, RAC, RAC-ALPHA}
- **Diseases:** hepatocellular carcinoma (MESH:D006528), esophageal cancer (MESH:D004938), neurofibromas (MESH:D009455), liver (MESH:D017093), dizziness (MESH:D004244), oral mucositis (MESH:D013280), liver metastasis (MESH:D009362), hypertension (MESH:D006973), medullary thyroid carcinoma (MESH:C536914), soft tissue sarcoma (MESH:D012509), non-small cell lung cancer (MESH:D002289), melanotic nerve sheath tumor (MESH:D018317), hypoxia (MESH:D000860), MMNST (MESH:D019574), nausea (MESH:D009325), fatigue (MESH:D005221), Cancer (MESH:D009369), lung lesions (MESH:D008171), weakness (MESH:D018908), sensory abnormalities (MESH:D012678), hand-foot syndrome (MESH:D060831), MS (MESH:D009442), neck mass (MESH:D006258), metastatic lesion (MESH:D000092182), MPNST (MESH:D018319), malignant melanoma (MESH:D008545), small cell lung cancer (MESH:D055752), pain (MESH:D010146)
- **Chemicals:** Anlotinib (MESH:C000625192), ifosfamide (MESH:D007069), sintilimab (MESH:C000632826), melanin (MESH:D008543), etoposide (MESH:D005047), cisplatin (MESH:D002945), denosumab (MESH:D000069448), doxorubicin (MESH:D004317), hematoxylin (MESH:D006416), everolimus (MESH:D000068338), bevacizumab (MESH:D000068258), triglyceride (MESH:D014280), fotemustine (MESH:C054368)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Figures

4 figures with captions in the complete paper: https://tomesphere.com/paper/PMC12909216/full.md

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Source: https://tomesphere.com/paper/PMC12909216