Final Diagnosis -- Castleman's Disease

FINAL DIAGNOSIS:   Castleman's disease, hyaline vascular type

DISCUSSION:

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.

Table 1   Summary of reports of Castleman's disease at an intercostal space

Authors	Age and Sex	Location, Size	Time, Diagnosis/ Surgery	Pathology	Follow-up
Matsuda, 1988	69, Male	Left lateral chest wall, 2nd ICS†, 5.5cm	9 years, increase in size	CD, HV with encapsulation	Alive and well at 3 yrs
Parrot, 1989	42, Female	Anterior chest wall, 2nd IC‡, 6cm	Immediate removal	CD, HV with encapsulation	None
Our Case, 1997	32, Female	Right 3rd IV§ foramen, 3cm	5 yrs Increase in size, discomfort	CD,HV nerve destruction	Alive and well at 2 yrs

†ICS: Intercostal Space, ‡IC: Intercostal, §IV: Intervertebral, CD,HV: Castleman's Disease, Hyaline Vascular Type

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).

References

1. Castleman B, Iverson L, Menendez P. Localized mediastinal lymph node hyperplasia resembling thymoma. Cancer (1956) 9:822-30.
2. Chen TC, Kuo T. Castleman's disease presenting as a pedunculated nasopharyngeal tumor simulating angiofibroma. Histopathol (1993) 23: 485-88.
3. Danon AD, Krishnan J, Frizzera G. Morpho-immunophenotypic diversity of Castleman's disease hyaline vascular type: with emphasis on a stroma rich variant and new pathogenetic hypothesis. Virch Arch (1993) 423: 369-82.
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.
5. Gerald W, Kostianovsky M, Rosai J. Development of vascular neoplasia in Castleman's disease: Report of seven cases. Am J Surg Path. (1990) 14:603-14.
6. Matsuda H, Mori M, Yasumoto K, Sugimachi K. Angiofollicular lymph node hyperplasia arising from the intercostal space. Thorax (1988) 43:337-338.
7. Parrot A, Bazelly B, Milleron BJ, Carette MF, Georges O, Akhoun GM. Unusual thoracic localization of Castleman's Tumor. Rev pneumolo clini (1989) 45:168-70.
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