2024 Asia Conference on Lung Cancer (ACLC) - Poster & ePosters-PP01.50

PP01.50 - Sari Yasuta

Pdf Summary

This case report from Yamagata University details the challenging diagnosis of unicentric Castleman disease (UCD) in a 24-year-old woman. Initially presenting with refractory stomatitis and genital ulcers in November 2022, her condition progressed to shortness of breath by March 2023. By August 2023, a CT scan revealed an 11 cm anterior mediastinal tumor. Additional complications included severe mixed ventilatory disturbance and bronchiolitis obliterans (BO). <br /><br />Despite high surgical risks and inconclusive CT biopsies, the patient was referred to the hospital in September 2023. Following surgical removal of the tumor via a median sternotomy, the pathological diagnosis confirmed hyaline vascular type Castleman disease, associated with paraneoplastic pemphigus (PNP) and BO. <br /><br />The postoperative course saw the successful removal of a chest drain on postoperative day one, and the patient was discharged by day six. By December 2023, she was admitted for PNP treatment, with gradual improvement allowing discharge by January 2024. Although her cutaneous symptoms improved, her respiratory function continued to decline, leading to her placement on a lung transplant waiting list for BO.<br /><br />Diagnosis of UCD can be challenging due to its diverse presentations and similarities to various thymic malignancies, particularly when associated with conditions such as PNP and BO which are linked to poor prognosis. This case underscores the importance of a multidisciplinary approach for effective diagnosis and treatment, highlighting that complete tumor resection reduces recurrence risk. It took 14 months from the initial symptom presentation to the final diagnosis, illustrating the diagnostic complexity of UCD, especially when complicated by PNP and BO. This case emphasizes considering mediastinal tumors, particularly UCD, in differential diagnoses for similar presentations.

Keywords

unicentric Castleman disease

UCD

paraneoplastic pemphigus

PNP

bronchiolitis obliterans

mediastinal tumor

multidisciplinary approach

diagnostic complexity

surgical resection