Palacios Huatuco René M, Pantoja Pachajoa Diana A, Pinsak Agustín E, Salvano Mario L, Doniquian Alejandro M, Mandojana Facundo I
Oncology Surgery Sector, General Surgery Service, Clínica Universitaria Reina Fabiola, Universidad Católica de Córdoba, Oncativo, 1248, Córdoba Capital, Argentina.
Oncology Surgery Sector, General Surgery Service, Clínica Universitaria Reina Fabiola, Universidad Católica de Córdoba, Oncativo, 1248, Córdoba Capital, Argentina.
Int J Surg Case Rep. 2020;77:803-808. doi: 10.1016/j.ijscr.2020.11.129. Epub 2020 Nov 30.
Primary mediastinal B-cell lymphoma (PMBCL) is an uncommon subtype of non-Hodgkin lymphoma (2-3%), predominantly occurring in female young adults. Extrathoracic involvement is found in 10-20%. It can affect the kidneys, pancreas, stomach, adrenal glands, liver, and infrequently the central nervous system (6-9%). There is currently only one reported case of ileum dissemination with a single perforation.
A 51-year-old woman with a history of PMBCL, hospitalized by a superior vena cava syndrome. PET-CT showed numerous lesions in the small intestine, pancreas, adrenal glands, and left kidney. During chemotherapy she presented abdominal symptoms, requiring an emergency laparotomy. On examination, six perforation sites were found in the small intestine. The pathology report revealed lesions compatible with PMBCL spread.
There are few case series with reports of dissemination in the gastrointestinal tract, with the main location in the stomach. Knowing the visceral location of the PMBCL would allow us to plan a strict follow-up during the first phases of chemotherapy treatment, as well as the early diagnosis of unexpected complications, such as intestinal perforation.
The PMBCL is a rare entity. Visceral involvement should be suspected in these patients since intestinal perforation represents a complication with high morbidity and mortality. This is the first case reported with numerous intestinal locations and multiple post-chemotherapy perforations.
原发性纵隔B细胞淋巴瘤(PMBCL)是非霍奇金淋巴瘤中一种不常见的亚型(2%-3%),主要发生于年轻女性。10%-20%的患者存在胸外受累情况。它可累及肾脏、胰腺、胃、肾上腺、肝脏,很少累及中枢神经系统(6%-9%)。目前仅有一例回肠播散伴单个穿孔的报道。
一名51岁有PMBCL病史的女性因上腔静脉综合征入院。PET-CT显示小肠、胰腺、肾上腺及左肾有多处病灶。化疗期间她出现腹部症状,需要紧急剖腹探查。检查发现小肠有六个穿孔部位。病理报告显示病灶与PMBCL播散相符。
关于胃肠道播散的病例系列报道较少,主要部位在胃。了解PMBCL的内脏受累情况有助于我们在化疗治疗的早期阶段规划严格的随访,并对意外并发症如肠穿孔进行早期诊断。
PMBCL是一种罕见疾病。这些患者应怀疑有内脏受累,因为肠穿孔是一种发病率和死亡率都很高的并发症。这是首例报道的有多处肠道受累及化疗后多处穿孔的病例。