Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) is an indolent non-Hodgkin lymphoma rarely seen in pediatric patients. MALT lymphoma most commonly involves the gastrointestinal tract or peri-orbital tissues, potentially as sequela of chronic antigenic stimulation or immune dysregulation. Rare cases of MALT lymphoma arising from the gynecologic tract have been reported in older adult patients. We present the unique case of a 16-year-old postpubescent female with MALT lymphoma localized to the gynecologic tract, who initially presented with abdominal fullness, abnormal uterine bleeding, and obstructive acute kidney injury secondary to urinary outflow obstruction. Intraoperatively, dense fibrosis of the uterus and left fallopian tube was noted which mimicked abdominal cocoon syndrome. She was treated with 6 cycles of bendamustine and rituximab with complete anatomic and metabolic remission. In this report we highlight a very unusual presentation of a rare malignancy in the pediatric population as well as unique treatment considerations given this patient's young age and tumor location.

中文翻译：

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青少年患者结外边缘区淋巴瘤类似腹部茧综合征的罕见病例。

粘膜相关淋巴组织的结外边缘区淋巴瘤（MALT 淋巴瘤）是一种惰性非霍奇金淋巴瘤，在儿科患者中很少见。MALT 淋巴瘤最常累及胃肠道或眼眶周围组织，可能是慢性抗原刺激或免疫失调的后遗症。老年患者中已有罕见的妇科 MALT 淋巴瘤病例报告。我们介绍了一名 16 岁青春期后女性的独特病例，患有局限于妇科的 MALT 淋巴瘤，她最初表现为腹部饱胀、子宫异常出血和继发于尿流梗阻的梗阻性急性肾损伤。术中发现子宫和左侧输卵管致密纤维化，类似于腹部茧综合征。她接受了 6 个周期的苯达莫司汀和利妥昔单抗治疗，解剖和代谢完全缓解。在本报告中，我们强调了儿科人群中一种罕见恶性肿瘤的非常不寻常的表现，以及考虑到该患者的年轻年龄和肿瘤位置的独特治疗考虑因素。