Brain Pathology Case of the Month - March 2002

FINAL DIAGNOSIS:   Castleman's disease, hyaline vascular type


The postero-superior mediastinum is an exceedingly rare location for the Castleman's Disease, hyaline vascular type. It is likely that this mass arose from one of the posterior intercostal lymph nodes, situated in the paravertebral region, however the capsule was not readily seen and the sinuses were not apparent. No etiology was found, there was no autoimmune disease and no immunodeficiency syndrome. She recovered well following the surgery and was well and alive two years later, having resumed her daily activities.

Almost all previous cases of Castleman's Disease, hyaline vascular type were described in the anterior mediastinum. Two cases were reported, at one lateral intercostal space, mimicking a chest wall tumor, and arising from an intercostal lymph node (6,7) (Table 1). Both reports mentioned the encapsulation of the lesion and the lack of involvement of neighboring structures.

Several previous reports have demonstrated that Castleman's disease can mimic various tumors, for example: thymoma (1), angiofibroma (2), meningioma (8), pulmonary neoplasms (9), adnexal mass (10), and cervical mass (4). Many lesions exhibit a similar radiological appearance and only a pathologic examination allows an accurate diagnosis. Because Castleman's disease is a rare reactive entity, its diagnosis is generally overlooked by radiologists and clinicians.

In hyaline vascular Castleman's disease, the adherence by an involved lymph node to the adjacent soft tissue has been described (10), however encapsulation and self-limitation are constant features of hyaline vascular Castleman's disease. Hyaline vascular Castleman's disease usually does not invade and replace neighboring structures. Our case is unique because it exhibits local invasion of adjacent structures and questions the possible aggressiveness of the hyaline vascular type, classically considered a hamartomatous or inflammatory lesion rather than a neoplasm. Surgery (which is curative) is the preferred treatment over close observation, in all cases.

In 1990, Rosai (5) suggested that the onset of Kaposi sarcoma-like neoplasms in hyaline vascular Castleman's disease could be mediated by the production of angiogenic factors by the activated lymphoid cells. In 1993, Frizzera (3) commented that the factors responsible for angiogenesis and the cellular growths that characterize hyaline vascular Castleman's disease remain to be determined. We think that angiogenesis is probably one determining factor in the local invasiveness seen here. More observations are needed to understand the potential behavior of the hyaline vascular type.

The patient is free of recurrence 7 years after the excision. After this case was accepted for this series, there have been two reports of Castleman's Disease in the posterior fossa (11, 12).


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  2. Chen TC, Kuo T. Castleman's disease presenting as a pedunculated nasopharyngeal tumor simulating angiofibroma. Histopathol (1993) 23: 485-88.
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  4. el Messaoudi A, el Edghiri H, Lazrak A, Jazouli N, Kzadri M. Castleman's disease: A propos of two cervical localizations. Rev Laryngolo Octolo Rhinolo (1993) 114:189-91.
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  8. Severson GS, Harrington DS, Weisenburger DD, et al. Castleman's disease of the meninges. J Neurosurg (1988) 69:283-6.
  9. Spedini C, Lombardi C, Lanzani G, Di Fabio D, Chiodera PL. Castleman;s disease presenting as a asympotomatic solitary pulmonary nodule. Monaldi Arch Chest Dis (1995) 50:363-365.
  10. Ylinen K, Sarlomo-Rikala M, Laatikainen T. Pelvic Castleman's disease mimicking an adnexal tumor. Obst Gyn (1995) 85:894-7.
  11. Iyoda A, et al. Castleman's Disease of the posterior mediastinum. Surg Today (2000) 30:473-476.
  12. Pereira TC, et al. Large posterior mediastinal mass in a young woman. Arch Pathol Lab Med (2001) 125:964-967

Contributed by Pascale Hummel MD, Vallo Benjamin MD, David Zagzag MD, PhD

International Society of Neuropathology