罕見後腹腔低惡性度B細胞淋巴瘤病例合併副腫瘤性天皰瘡和細支氣管炎的肺部疾病

尤仲豪醫師¹, 卓育慶醫師¹

¹國防醫學院三軍總醫院外科部泌尿外科

A rare of retroperitoneal low-grade B‐cell lymphoma associated with paraneoplastic pemphigus and bronchiolitis of lung disease

Chung-Hao Yu, MD¹, Yu-Chin Juho, MD¹

¹Division of Urology, Department of Surgery, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan

Abstract

Introduction:

Retroperitoneal lymphoma is rare of retroperitoneal tumors. Clinical diagnosis of retroperitoneal tumors is often delayed, because of asymptomatic until they become very large (range 15-18 cm). The gold standard for diagnosis is contrast enhanced Abdominopelvic CT Scan. Safe image guided biopsy or surgical biopsy can be arranged for provement.

Paraneoplastic pemphigus (PNP) is an autoimmune disorder, it occur at an underlying neoplasm in which patients have polymorphous skin and mucous membrane lesions.

Patient concerns:

A 58 years old female had general weakness and short of breath since Oct. 2020, so she went to CHE OPD for evaluate. Body weight loss about 12 kg in 4 months, and oral ulcers (Lichen Planus) was also found. Chest CT revealed bronchiolitis obliterans without lung cancer, but retroperitoneal tumor was found incidentally. Thus, abdominal CT was performed, heterogenous, huge, retroperitoneal tumor lesion (about 12cm) occupied inferior vena cava and left renal pedicle. The patient refer to GU for further management. We consult DER specialist for further management. Dermatologist found mucositis over lips and non-specific eczematous papules on right arm, paraneoplastic pemphigus was highly suspect. Low-grade B cell lymphoma (atypical lymphoid hyperplasia) was finally showed by sono-guided biopsy.

Interventions:

Corticosteroid for control symptoms.

Outcomes:

Dyspnea, weakness were got improved, pemphigus was subsided.  

Discussion:

Eczematous papules, dyspena, weight loss, fatigue was revealed at patient. Lymphoma must to be considered, the clinician need to arrange chest and abdominal image study for further survey.