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Neuroradiology |
1 From the Departments of Radiology (R.J.W., M.H.L.) and Pathology (J.W.M.), University of Virginia, Charlottesville, Va. Received December 23, 1998; revision requested February 2, 1999; revision received February 16; accepted June 8. Address reprint requests to R.J.W., Department of Radiology, Emory University Hospital, 1364 Clifton Rd, NE, Atlanta, GA 30322. (e-mail: rjwoodcock@hotmail.com).
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Abstract |
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Index terms: Histiocytosis, 145.66 • Pituitary, abnormalities, 145.66 • Pituitary, MR, 145.121411 • Sella turcica, 122.66
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Introduction |
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Case Report |
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At the present admission, she showed no systemic symptoms. Neurologic examination results were normal, and no hepatosplenomegaly or lymphadenopathy was present. MR imaging (Vision; Siemens, Ehrlangen, Germany) at 1.5 T demonstrated enlargement of the pituitary infundibulum (Fig 1) with extension of soft tissue into the suprasellar cistern, around the optic chiasm and lamina terminalis. The soft tissue was isointense to gray matter on T1- and T2-weighted images. Heterogeneous, mild enhancement was seen. She underwent a biopsy by means of a subfrontal approach, and histologic examination showed polymorphic histiocytic and lymphocytic infiltrate with prominent lymphophagocytosis, which is consistent with a diagnosis of SHML (Fig 2). No eosinophils or Birbeck granules, normally found in Langerhans cell histiocytosis, were present. The cells were focally immunopositive for S-100. Treatment with steroids was instituted, and follow-up MR imaging 6 months after biopsy demonstrated no change in the findings. However, follow-up examination at 9 months showed slight interval increase in the size of the lesion.
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Discussion |
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SHML most commonly affects otherwise healthy individuals in the first and second decades of life; mean age of onset is 20.6 years (4). There is a slight male predominance. Eighty-three percent of patients have painless, bilateral, massive cervical adenopathy at the time of diagnosis; however, as 17% do not have adenopathy, this is not necessary for the diagnosis (4). Four percent demonstrate neurologic manifestations (13). However, to our knowledge, symptoms of hypothalamic-pituitary axis dysfunction have been described in only one case, in which diabetes insipidus was present (14). No cases of intracranial involvement with anterior pituitary dysfunction, as is present in this case, were found in our review. Adenopathy at sites other than in the cervical region is reported as a less frequent occurrence. Systemic symptoms of fever and weight loss are frequently present (1–3,7). Laboratory study results are nonspecific, with an elevated erythrocyte sedimentation rate in a majority of cases. A mild normocytic or microcytic anemia also may be present, and leukocytosis is an inconsistent finding (3,7). Hypergammaglobulinemia and other immunologic manifestations may be present (15).
The disease has a distinctive histologic appearance on which the diagnosis is based. In nodal disease, pericapsular fibrosis and marked dilatation of subcapsular and medullary sinuses is present. In both nodal and extranodal disease, there are abundant histiocytes and lymphocytes with occasional plasma cells. Emperipolesis (lymphophagocytosis) is a nonspecific but sensitive finding that also occurs in hemophagocytic lymphohistiocytosis (7,16). Electron microscopy reveals a notable absence of Birbeck granules, as seen in Langerhans cell histiocytosis (7). The histiocytes of SHML are immunoreactive with S-100, an antigen found in normal antigen-presenting cells and in some abnormal histiocytes, which distinguishes these from histiocytes found in granulomatous disease (11,17).
No specific therapy is available, and treatment has included surgery, radiation therapy, and chemotherapy (6,7,11,16,18). Some authors (16,18) have advocated treatment with steroids or immunosuppressants, which have shown a variable response. The disease most often has a chronic, relapsing course, although fatal cases have been reported (19,20).
Extranodal involvement occurs in more than 40% of patients, with the most common sites being paranasal sinuses, orbit, spine, skull base, skin, and upper respiratory tract (3–5,8,10,20,21). Involvement of the central nervous system is uncommon, occurring in 22% of patients, with isolated disease occurring only rarely. In more than half of the reported cases, a second site of disease was present (7,13). Disease of the central nervous system typically manifests in the epidural or subdural compartments. Intracranial involvement is twice as common as spinal involvement (3,6,9–11). With involvement of the central nervous system, there is a reported increase in immunologic abnormalities—47% in cases with involvement of the central nervous system versus 22% in those without such involvement—which portends a poorer prognosis (4).
Reports (11,15) of CT findings of disease of the central nervous system have described dural-based masses that may be hyperattenuating, demonstrate enhancement, and be associated with bone erosion. The lesions are angiographically avascular (11,15). MR imaging findings of SHML in the orbit and posterior fossa have been described; the lesions were isointense to white matter on T1-weighted images in the posterior fossa and isointense to gray matter on T1-weighted images in the orbit (21).
Suprasellar involvement has been reported in three cases, only one of which was isolated to this site alone, as in our case (8,12,14). In two cases, the suprasellar mass was seen in association with multiple extranodal sites of involvement; diagnosis was made at autopsy in one case and at CT in the other (8,14). In the third case, a 78-year-old man presented with visual impairment, and an enhancing mass extending from the sella turcica along the planum sphenoidale was seen at CT; diagnosis was made at surgery (12). No reports of MR imaging findings of suprasellar disease were found in our review of the literature. A single case of isolated sellar SHML had a posterior sellar mass with diminished enhancement relative to the pituitary gland on MR images; diagnosis was made following fine-needle aspiration (14).
The differential diagnosis in this case included suprasellar germinoma, granulomatous disease, other histiocytoses (Langerhans cell histiocytosis and hemophagocytic lymphohistiocytosis), and metastasis. Germinoma in the suprasellar region occurs with equal frequency in male patients and female patients, unlike in the pineal region where it is seen more often in male patients, and is typically an infiltrating mass that is isointense to gray matter on T1-weighted images and hyperintense on T2-weighted images (22). Homogeneous enhancement is typical (8). These findings are similar to those seen in this case. Histologically, however, large cells with large nuclei (germ cells) are identified (23).
Langerhans cell histiocytosis has variable manifestations in the central nervous system that range from localized enhancing masses to lytic osseous lesions. Whereas some histologic features are shared between Langerhans cell histiocytosis and SHML, there are several distinguishing features. In Langerhans cell histiocytosis, the histiocytes do not exhibit lymphophagocytosis or erythrophagocytosis, and eosinophils are conspicuously present. Moreover, electron microscopy often reveals the pathognomonic Birbeck granules (7). Hemophagocytic lymphohistiocytosis is a chronic, progressive disorder characterized by fever, hepatosplenomegaly, and hematopoietic deficits, and these features are absent in this case (7). Histologically, however, it may be similar to SHML. Granulomatous diseases (tuberculosis and sarcoidosis) are distinguished histologically by the presence of granulomas with or without caseation and organisms (22,23). Metastasis may be distinguished by multiplicity of lesions and a clinical history of a primary site.
This case report is the first, to our knowledge, to characterize SHML (Rosai-Dorfman disease) in the suprasellar region on MR images; furthermore, the case history of anterior pituitary dysfunction is unique. Whereas this is an unusual manifestation of a rare disorder, it is an important entity in that it may mimic other lesions, particularly other histiocytic disorders. Sinus histiocytosis should be considered in the evaluation of a suprasellar lesion, especially if massive lymphadenopathy or histiocytic histologic findings are present.
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Footnotes |
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Author contributions: Guarantor of integrity of entire study, R.J.W.; study concepts, R.J.W., J.W.M.; study design, R.J.W., J.W.M., M.H.L.; definition of intellectual content, R.J.W., J.W.M.; literature research, R.J.W.; clinical studies, R.J.W.; data acquisition, R.J.W.; manuscript preparation, R.J.W.; manuscript editing, R.J.W., J.W.M., M.H.L.; manuscript review, R.J.W., M.H.L.
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