We report the case of a 36-year-old woman who presented with headache, fever, and amenorrhea. Laboratory analysis revealed hypopituitarism and autoimmune thyroiditis, while a cerebrospinal fluid study suggested concurrent aseptic meningitis. A magnetic resonance image (MRI) scan revealed a 1.0x0.9 cm cystic mass enlarging the sella turcica. Surgical resection via an endoscopic transsphenoidal route was performed. The histological finding of the excised tissue revealed foamy histiocytes with vacuolated cytoplasm, supporting the diagnosis of xanthomatous hypophysitis. Although a residual soft lesion was observed on the MRI image postoperatively, the patient's headache and fever improved. Ten months after surgery, the patient complained of visual impairment and headache, and the residual mass had enlarged into the suprasellar area. High dose (500 mg intravenous) methylprednisolone was administered for 3 days. During the methylprednisolone pulse therapy, the patient's visual acuity and headache improved. A follow-up MRI taken after methylprednisolone therapy showed a marked mass reduction. Our case supports an autoimmune pathophysiology for xanthomatous hypophysitis and suggests that high dose glucocorticoid therapy as a treatment option.
Amenorrhea ; Cytoplasm ; Female ; Fever ; Follow-Up Studies ; Glucocorticoids ; Headache ; Histiocytes ; Humans ; Hypopituitarism ; Magnetic Resonance Spectroscopy ; Meningitis, Aseptic ; Methylprednisolone ; Sella Turcica ; Thyroiditis, Autoimmune ; Vision Disorders ; Visual Acuity

BACKGROUND: POEMS syndrome is a rare paraneoplastic syndrome associated with plasma cell dyscrasia. High-dose chemotherapy followed by autologous stem cell transplantation has shown encouraging efficacy in the treatment of patients with POEMS syndrome. However, there are minimal reports on clinical outcomes after autologous stem cell transplantation for patients with advanced disease and very poor performance status. METHODS: We retrospectively evaluated 9 advanced POEMS syndrome patients, who had an Eastern Cooperative Oncology Group performance status score of 3 or 4, and were treated with high-dose melphalan therapy followed by autologous stem cell transplantation from 2004 to 2011. RESULTS: Eight patients achieved initial hematologic response, 4 of whom had complete responses. At a median follow-up of 44 months (range, 8-94 months), 7 patients were alive, with 3-year overall survival rate of 77.8%. There were no hematologic relapses in the survivors. One patient died of disease progression; the other died of pneumonia despite a hematologic response 3 months after autologous stem cell transplantation. All survivors achieved improvement in general performance status and in clinical response. CONCLUSION: High-dose melphalan followed by autologous stem cell transplantation can be considered a valid treatment option even for patients with advanced POEMS syndrome.
Disease Progression ; Drug Therapy ; Follow-Up Studies ; Humans ; Melphalan* ; Paraneoplastic Syndromes ; Paraproteinemias ; Pneumonia ; POEMS Syndrome* ; Recurrence ; Retrospective Studies ; Stem Cell Transplantation* ; Stem Cells* ; Survival Rate ; Survivors

The buccal fat pad is specialized fat tissue located anterior to the masseter muscle and deep to the buccinator muscle. Possessing a central body and four processes it provides separation allowing gliding motion between muscles, protects the neurovascular bundles from injuries, and maintains facial convexity. Because of its many advantageous functions, the use of the buccal fat pad during oral and maxillofacial procedures is promoted for the reconstruction of defects secondary to tumor resection, and those defects resulting from oroantral fistula caused by dento-alveolar surgery or trauma. We used the pedicled buccal fat pad in the reconstruction of intraoral defects such as oroantral fistula, maxillary posterior bone loss, or defects resulting from tumor resection. Epithelization of the fat tissue began 1 week after the surgery and demonstrated stable healing without complications over a long-term period. Thus, we highly recommend the use of this procedure.
Adipose Tissue ; Masseter Muscle ; Muscles ; Oral Surgical Procedures ; Oroantral Fistula