Flauto Fabiano, Servetto Alberto, Bianco Roberto, Formisano Luigi
Department of Clinical Medicine and Surgery, University of Naples Federico II, Naples, Italy.
Front Oncol. 2024 Sep 20;14:1465130. doi: 10.3389/fonc.2024.1465130. eCollection 2024.
Granular Cell Tumors (GCTs), also known as Abrikossoff tumors, are rare neoplasms that typically originate from Schwann cells. These tumors most commonly occur in the head and neck region, particularly the tongue. While GCTs are generally benign, less than 2% of cases exhibit aggressive biological features such as rapid growth, high recurrence rates, and metastasis. In this report, we present a rare case of a Malignant Granular Cell Tumor (MGCT) of the chest wall, which posed significant challenges in both characterization and management.
A fifty-year-old man underwent an ultrasound examination for a nodular mass on his right chest wall. The ultrasound revealed a firm, hard mass measuring 2 cm x 2 cm with an uncertain diagnosis. A fine-needle aspiration biopsy (FNAB) guided by ultrasound was performed, resulting in a diagnosis of Abrikossoff tumor. The patient subsequently underwent radical excision of the mass, which confirmed the initial diagnosis. Nine months after surgery, a new mass with similar characteristics was detected during a physical examination. The patient underwent a second surgery, but this time the histopathological examination was negative for neoplastic cells. However, another mass appeared at the same site as the previous surgical excision. A CT scan and MRI of the right chest wall confirmed the presence of a 2 cm x 2 cm nodular mass. The patient then underwent a deeper excision. Histomorphological and immunohistochemical assessments confirmed the recurrence of MGCT.
This case highlights the malignant potential of GCTs. The numerous local recurrences necessitated three surgeries and additional procedures. The aggressive nature of this pathology underscores the complexity of managing these tumors, which are poorly understood and lack proven post-operative strategies for controlling local and distant disease.
颗粒细胞瘤(GCTs),也称为阿布里科索夫瘤,是一种罕见的肿瘤,通常起源于施万细胞。这些肿瘤最常发生在头颈部区域,尤其是舌头。虽然颗粒细胞瘤一般为良性,但不到2%的病例表现出侵袭性生物学特征,如快速生长、高复发率和转移。在本报告中,我们介绍了一例罕见的胸壁恶性颗粒细胞瘤(MGCT)病例,该病例在特征描述和治疗方面都带来了重大挑战。
一名50岁男性因右胸壁出现结节性肿块接受了超声检查。超声显示一个质地坚硬的2厘米×2厘米肿块,诊断不明确。在超声引导下进行了细针穿刺活检(FNAB),诊断为阿布里科索夫瘤。患者随后接受了肿块根治性切除术,证实了最初的诊断。手术后九个月,体检时发现了一个具有相似特征的新肿块。患者接受了第二次手术,但这次组织病理学检查未发现肿瘤细胞。然而,在先前手术切除部位又出现了一个肿块。右胸壁的CT扫描和MRI证实存在一个2厘米×2厘米的结节性肿块。患者随后接受了更深层次的切除。组织形态学和免疫组化评估证实了恶性颗粒细胞瘤的复发。
本病例突出了颗粒细胞瘤的恶性潜能。多次局部复发需要进行三次手术及其他治疗。这种病理的侵袭性凸显了管理这些肿瘤的复杂性,目前对这些肿瘤了解不足,且缺乏行之有效的术后控制局部和远处疾病的策略。