Idiopathic hypertrophic pachymeningitis resulting in delayed panhypopituitarism.

OBJECTIVE: To describe an adult man with idiopathic hypertrophic pachymeningitis (IHPM) that progressed to involve the pituitary gland and caused hypopituitarism 6 years after the onset of the first symptom. METHODS: We describe the slow 6-year progression of IHPM in a man being treated with steroids, radiation, and antineoplastic medications and present clinical, pathologic, and imaging data. The pertinent literature is also reviewed. RESULTS: A 35-year-old man who presented with headaches, pain, and sensory loss on the right side of his face had thickened, inflamed dura without granulomas. Initially, the right middle fossa, the lateral wall of the right cavernous sinus, and the tentorium were involved. Results from a dural biopsy specimen were consistent with IHPM. His symptoms were only partially controlled with continuous high-dose steroids, cyclophosphamide, azathioprine, and radiation therapy. IHPM was diagnosed based on findings from an open brain biopsy. Other pathologic causes of inflammatory dural thickening were excluded. Six years after the onset of the first clinical manifestation of IHPM, the patient developed panhypopituitarism. Magnetic resonance imaging showed that the inflammatory process had invaded the pituitary gland. IHPM was confirmed by findings from transsphenoidal biopsy. CONCLUSION: IHPM is a rare disorder characterized by an unexplained inflammatory thickening of the dura at the skull base. The usual clinical symptoms are intractable headache and cranial nerve palsies. Physicians must be aware that IHPM can be slowly progressive despite attempted treatment, and that the disease process can invade the sella with dysfunction of both the anterior and the posterior pituitary gland.

Atrial plasmacytoma and hypercalcemia.

OBJECTIVE: To describe a patient with hypercalcemia due to multiple myeloma and an associated atrial plasmacytoma. METHODS: A case report is presented with clinical and laboratory findings. We review the differential diagnosis of hypercalcemia and discuss multiple myeloma and plasmacytomas. RESULTS: A 48-year-old woman presented to the emergency department with lethargy after sustaining trauma to the head. She was found to have a subdural hematoma and a parietal intraparenchymal hemorrhage. She had a serum calcium level of 17.2 mg/dL (normal, 8.4 to 10.6), anemia, thrombocytopenia, and renal insufficiency. Serum protein electrophoresis revealed a monoclonal gammopathy, and a bone marrow biopsy specimen was consistent with multiple myeloma. The patient required surgical evacuation of her subdural hematoma. Postoperatively, multiple complications developed, including a supraventricular tachycardia. She did not regain consciousness, and ventilatory support was withdrawn. On autopsy, she was found to have disseminated myeloma and an atrial plasmacytoma. CONCLUSION: This case report emphasizes the need to consider a plasmacytoma as a manifestation of multiple myeloma when localizing symptoms are present.

Langerhans' cell histiocytosis involving the pituitary, thyroid, lung, and liver.

OBJECTIVE: To report the development, in an adult patient, of Langerhans' cell histiocytosis (LCH) involving the thyroid and most probably the pituitary gland, lungs, and liver. METHODS: We present a case report of a 29-year-old woman who requested a medical consultation because of polyuria and was found to have pituitary dysfunction. We describe the subsequent follow-up, which revealed progressive liver disease that necessitated transplantation and also the presence of a goiter, and discuss the unpredictable pathologic features of LCH. RESULTS: After the diagnosis of central diabetes insipidus and partial hypopituitarism, the patient was diagnosed 2 years later with sclerosing cholangitis. The hepatic involvement was progressive, and she required transplantation and immunosuppression. Three months before liver transplantation, a goiter was discovered. Fine-needle aspiration of the thyroid revealed infiltration by LCH. The goiter decreased in size after chemotherapy with vinblastine and prednisone. Computed tomography of the chest showed bilateral thin-walled cysts, consistent with eosinophilic granulomas. CONCLUSION: In patients with central diabetes insipidus and pituitary stalk thickening on imaging studies, LCH should be considered in the differential diagnosis. Other hormonal deficiencies may be present initially or may evolve after many years. Thus, continual surveillance is necessary. In addition, an ongoing potential exists for involvement of other organ systems such as the thyroid, liver, and lungs. We suggest consideration of LCH as a possible cause of a goiter in such patients. In our patient, it remains to be seen what effect the immunosuppressive therapy for the liver transplant has on the LCH disease process.