Printes Thaís Regina Moreira, de Castro Gustavo Lopes, Rabelo Delon Calixto, da Silva Neto Rubem Alves, da Silva Junior Rubem Alves
Digestive Surgery Service at Getúlio Vargas University Hospital (HUGV).
Federal University of Amazonas' (UFAM) Medical School, Manaus, Amazonas, Brazil.
Ann Med Surg (Lond). 2024 Jun 17;86(8):4861-4864. doi: 10.1097/MS9.0000000000002273. eCollection 2024 Aug.
Solid pseudopapillary tumor is a low-grade malignancy of the pancreas and predominantly affects young women. This neoplasm is a rare pancreatic entity with vague clinical presentation. Diagnosis is often incidental through imaging or even during surgical approach for another condition.
A 22-year-old Brazilian female with gastrointestinal symptoms was diagnosed with achalasia and underwent Heller myotomy. Intraoperatory findings included an enlarging mass in the distal pancreas. During follow-up for the surgical approach of achalasia, a hypothesis of Frantz's tumor was stated, and spleen-preserving distal pancreatectomy was performed.
The pathological pathways of Frantz's tumor is still unclear, and its connection with chromosomal abnormalities is under investigation. Although the tumor has been reclassified over the years to solid pseudopapillary tumor, surgical resection remains the standard treatment.
Despite a surgical challenge, surgery presents a great prognosis in these patients and long-term survival. High suspicion and proper investigation are fundamental to diagnosis and early treatment.
实性假乳头状肿瘤是一种低度恶性的胰腺肿瘤,主要影响年轻女性。这种肿瘤是一种罕见的胰腺病变,临床表现不明确。诊断通常是通过影像学偶然发现的,甚至是在因其他疾病进行手术时发现的。
一名22岁有胃肠道症状的巴西女性被诊断为贲门失弛缓症并接受了贲门肌切开术。术中发现包括胰腺远端有一个增大的肿块。在对贲门失弛缓症手术进行随访期间,提出了弗兰茨肿瘤的假说,并进行了保留脾脏的远端胰腺切除术。
弗兰茨肿瘤的病理途径仍不清楚,其与染色体异常的关系正在研究中。尽管多年来该肿瘤已被重新分类为实性假乳头状肿瘤,但手术切除仍然是标准治疗方法。
尽管手术具有挑战性,但手术对这些患者的预后和长期生存具有重要意义。高度怀疑和适当的检查对诊断和早期治疗至关重要。