Department of Pathology and Laboratory Medicine, North Shore University Hospital and Long Island Jewish Medical Center, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell Health, 2200 Northern Blvd, Suite 104, Greenvale, NY, 11548, USA.
Department of Otolaryngology, Head and Neck Surgery, Long Island Jewish Medical Center, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell Health, New Hyde Park, NY, USA.
J Med Case Rep. 2024 Jan 20;18(1):24. doi: 10.1186/s13256-023-04280-7.
Carcinosarcoma of the parotid gland is an extremely rare malignancy comprising of 0.04-0.16% of all salivary gland tumors. This is the first case of an adenoid cystic carcinoma with chondrosarcoma to the best of our knowledge. They consist of distinct carcinomatous and sarcomatous components and may arise de novo or from a preexisting pleomorphic adenoma.
Herein we present a case of an 80-year-old white female who presented with progressively increasing left facial swelling over 6 weeks. Magnetic Resonance Imagining revealed a mass (3.4 cm) in the parotid gland with a predominant cystic/necrotic component. The cytology was atypical (Milan3) and a total parotidectomy and selective lymph node dissection was done. The resection showed extensive necrosis with high grade sarcomatous (chondrosarcoma) areas. The epithelial component was adenoid cystic carcinoma with perineural invasion. The patient is currently undergoing radiotherapy of the tumor bed and skull base due to propensity of perineural invasion of the adenoid cystic component. The most common carcinomas in carcinosarcomas of salivary glands are adenocarcinoma and squamous cell carcinoma.
Carcinosarcoma is a high-grade aggressive lesion with a poor prognosis and should be treated aggressively. More studies are needed to understand the origin of these tumors.
腮腺的癌肉瘤是一种极为罕见的恶性肿瘤，占所有唾液腺肿瘤的 0.04-0.16%。据我们所知，这是首例腺样囊性癌伴软骨肉瘤的病例。它们由明显的癌性和肉瘤性成分组成，可能是从头发生或从先前存在的多形性腺瘤发展而来。
在此，我们介绍一例 80 岁白人女性病例，她因左面部肿胀逐渐加重 6 周而就诊。磁共振成像显示腮腺内有一个肿块（3.4 厘米），主要为囊性/坏死性成分。细胞学为非典型（米兰 3 级），行全腮腺切除术和选择性淋巴结清扫术。切除标本显示广泛坏死伴高级别肉瘤（软骨肉瘤）区域。上皮成分是腺样囊性癌，伴有神经周围侵犯。由于腺样囊性成分有神经周围侵犯的倾向，患者目前正在接受肿瘤床和颅底的放射治疗。在唾液腺癌肉瘤中，最常见的癌是腺癌和鳞状细胞癌。
癌肉瘤是一种高级别侵袭性病变，预后不良，应积极治疗。需要更多的研究来了解这些肿瘤的起源。
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