In March 2012, a 42-year-old man was referred to our department for evaluation of whitish and erosive lesions that involved the tongue, buccal mucosa, palate, and lip. The patient previously received PBSCT for treatment of acute lymphocytic leukemia (ALL) in December 2005; his sister was the related donor, and she matched for HLA antigens. PBSCT was carried out after induction by combined chemotherapy and total body irradiation (TBI). The patient's ALL went into remission without evidence of acute GVHD. Cyclosporine A (100 mg) and prednisolone (10 mg) were utilized for posttransplantation GVHD prophylaxis. However, chronic GVHD (cGVHD) of the skin and eyes were developed 3 months after PBSCT. One month later, airflow obstruction developed, which was considered as evidence of BO. Later, white spots and erosions of the oral mucosa were noted, which persisted without significant change during follow-up until the first visit of our department. In August 2008, he became aware of respiratory discomfort due to pneumothorax of the right lung. The pneumothorax was successfully treated with a thoracic cavity drainage.
Intraoral examination revealed lichenoid changes and atrophic mucosa located on the buccal mucosa, tongue, and lip, compatible with oral cGVHD (Figure 1(a)). The patient was received plaque control instruction to improve poor oral hygiene associated with severe xerostomia. A lip biopsy for confirmation of oral cGVHD was performed on June 7, 2012. The biopsy specimen demonstrated a lichenoid infiltration compatible with cGVHD (NIH criteria: score 2).
In January 2013, 8 years after PBSCT, a raised mucosal lesion appeared on the left dorsal surface of the tongue base, with a background of lichenoid mucositis. This lesion decreased in size by 50% over the next month. However, the lesion then enlarged over the following 3 months and appeared as an exophytic mass (20 x 15 mm) (Figure 1(b)). The lesion was then biopsied; histopathologic examination showed well-differentiated OSCC (T2N0M0: stage II). After preoperative examination, we determined that the patient had adequate cardiorespiratory function to undergo an invasive operation under general anesthesia. In July 2013, hemiglossectomy and left supraomohyoid neck dissection and reconstruction with a rectus abdominis musculocutaneous flap were performed after cessation of immunosuppressants. Histopathologically, the excised specimen revealed a well-keratinized OSCC with negative tumor margins; regional lymph nodes were negative for metastasis. On the 7th day after the operation, the patient's respiratory status was suddenly deteriorated. Bilateral infiltrations were noted on plain chest radiography (Figure 2(a)), and significant ground-glass abnormalities were seen on a chest computed tomography (Figure 2(b)), indicating acute respiratory distress syndrome (ARDS). Subsequently, mechanical ventilation, intravenous antibiotics and sivelestat sodium, and control of body fluids were administered. The ARDS improved over two weeks. Adjuvant therapy for OSCC was not considered, given the patient's status. Although the surgical site remained clinically stable, approximately 2 months later, recurrent pneumothorax developed on the right. In October 2013, the patient was discharged after the pneumothorax improved. Thirteen months after surgery, the patient showed neither recurrence of tumor nor progression of oral GVHD. However, the patient died of respiratory failure due to repeated pneumothorax and deterioration of BO.