J Neurooncol (2008) 88:227–229 DOI 10.1007/s11060-008-9558-9
CLINICAL-PATIENT STUDIES
Extramedullary plasmacytoma presented as a non-functional invasive pituitary macro-adenoma Vincenzo Pitini Æ Carmela Arrigo Æ Concetta Alafaci Æ Giuseppe Altavilla
Received: 4 December 2007 / Accepted: 18 February 2008 / Published online: 4 March 2008 Ó Springer Science+Business Media, LLC. 2008
Abstract Pituitary adenomas are the most common etiology of sellar masses. Intra-sellar plasmacytomas are rare causes of sellar tumors of non-pituitary origin and may mimic nonfunctional pituitary adenomas clinically and radiologically. We report an uncommon case of an intrasellar plasmacytoma presenting as the only manifestation of multiple myeloma. Keywords Pituitary macro-adenoma Multiple myeloma Extramedullary plasmacytoma
Multiple myeloma (MM) involving the nervous system most commonly manifests as encroachment on nerve roots or compression of the spinal cord. Cranial and intracranial involvement by myelomatous disease is less frequently encoured and the majority of these cases had typical systemic manifestations of MM prior to the development of cranial and intracranial involvement. Here, we report a case of intrasellar plasmacytoma presenting as the first manifestation of multiple myeloma.
Case report A 65-year-old man was referred to our department with a diagnosis of a non-functional pituitary macro-adenoma. He
V. Pitini (&) C. Arrigo G. Altavilla Department of Medical Oncology (Pad. H 5° piano), University of Messina, Via Consolare Valeria, 98125 Messina, Italy e-mail:
[email protected] C. Alafaci Department of Neurosurgery, University of Messina, Messina, Italy
reported a 2-month history of headaches without symptoms of polydipsia, polyuria, fatigue, bone pain, back pain, galactorrhea, or visual changes. On physical examination a mild hypoaesthesia on the right hand side of face was detected. There were no systemic symptoms. Blood tests revealed normal biochemical and hematological parameters, endocrine evaluation was remarkable only for a modestly elevated serum prolactin 36.7 ng/ml (3–20 ng/ ml). A magnetic resonance imaging (MRI) scan of the brain revealed a homogenously enhancing intrasellar mass without evidence of extension into the nasopharynx and posterior nasal fossa (Fig. 1a, b). A transphenoidal biopsy of the mass was performed and the excised specimen consisted of multiple irregular red-brown soft tissue fragments of a cytological appearance of a highly cellular neoplasm composed of mature plasma cells including binucleate forms (Fig. 2). Immuno-cytochemistry showed that all the tumor cells were positive for CD 138 and kappa light chains confirming that this tumor was a plasmacytoma. Interphase fluorescence in situ hybridization combined with cytoplasmic light chain fluorescence detected a 13q14 deletion (probe LSIRB1-Vysis) in more than 15% of the nuclei (Fig. 3a), a p53 deletion (LSIp53 probe Vysis + 17-CEP alpha DNA centromeric probe) (Fig. 3b), and a monoclonal cytoplasmic expression of kappa light chains in malignant plasma cells. After biopsy an extensive investigation for a myelomatous disease was undertaken including serum and urine protein electrophoresis, a bone survey and a bone marrow biopsy without any evidence of an occult myelomatous disease, serum beta-2 microglobulin was normal. A final diagnosis of extramedullary plasmacytoma manifesting itself as a tumor involving the sella turcica without any systemic manifestations was made. He underwent tumoricidal radiation therapy directed to the tumor located in the sellar region using 5,000 cGy over a 5-week period.
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Fig. 2 Mature plasma cells with round eccentrically placed nuclei
Fig. 3 (a, b) Combined FISH and immunifluorescence staining of the cytoplasmatic kappa light chains. 13q14 deletion (a), p53 deletion (b)
Fig. 1 (a, b) Coronal and sagittal MRI scan. Homogenously enhancing intrasellar mass
A follow-up MRI at 4 months showed stabilization of the abnormality. Endocrine work-up did not demonstrate any evidence of evolving panhypopituitarism and a new extensive investigation for multiple myeloma was negative.
Discussion Plasma cell neoplasms include a variety of clinicopathologic entities including: multiple myeloma, solitary plasmacytoma of bone and extramedullary plasmacytoma, which are solitary lesions of a microscopic appearance of plasma cell neoplasms without any clinical or radiological evidence of multiple myeloma [1]. Extramedullary plasmacytoma must be distinguished from plasma cell
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granuloma and lymphoma (Malt, marginal zone and immunoblastic lymphomas). This is probably best accomplished by analysis of chromosomal abnormalities and phenotypic studies positive for CD 138 and a monoclonal cytoplasmic light chain expression of malignant plasma cells obtained by biopsy or fine needle aspiration of the solitary lesion. Approximately 85% of lesions occur in the head and neck mucosa, and underlying bone involvement, particularly in the sinuses may be noted [2]. The presentation of a plasma cell neoplasm as a cranial or intracranial tumor is an infrequent phenomenon and it is even more rare a presentation which mimics a pituitary adenoma [3]. In these cases it is believed that the origin of the tumor cells is the surrounding bone or the mucosa within the petrous or the sphenoid bone [4, 5]; however, even if a plasmacytoma is one of a wide variety of intrasellar masses which can mimic a pituitary adenoma, the differential diagnosis is broad and complex and the majority of reported cases had no known plasma cell tumor diagnosis until sellar biopsy, despite a careful endocrine workup, neurological and radiological assessment [6, 7].Recent studies with interphase fluorescent in situ hybridization indicate that all multiple myeloma cells harbor chromosome abnormalities [8]; monosomy of chromosome 13 representing 85% of them have an adverse prognosis in MM [9], likewise deletions of 17p13.1, the genomic locus of the p53 tumor
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suppressor gene have been associated with an adverse patient outcome. Curiously, even if the molecular genetic abnormalities in MM patients with CNS involvement have not been systemically studied the most frequent genetic changes in reported patients were deletions of 13q and p53 as also observed in our case [10].
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