Journal of Cancer Research and Therapeutics

: 2014  |  Volume : 10  |  Issue : 3  |  Page : 737--738

Unicentric Castleman's disease located in the scapular region

Abdul Rasheed, Ather Hafiz Khan, Mohsinul Rasool, Afiya Shafi 
 Department of Pathology, Sher-I-Kashmir Institute of Medical Sciences/Medical College, Bemina, Srinagar, Jammu and Kashmir, India

Correspondence Address:
Dr. Ather Hafiz Khan
Department of Pathology, Sher-I-Kashmir Institute of Medical Sciences/Medical College, Bemina, Srinagar, Jammu and Kashmir


Castleman«SQ»s disease (CD) or giant node hyperplasia is a rare benign disease of unknown etiology characterized by lymphoid hyperplasia. Although the most common site of the disease is the mediastinum, very few cases occurring in lymph nodes elsewhere have been reported in English literature, including only 10 intramuscular cases. We report a case of unicentric CD of the hyaline vascular type in a 30 year female located in the right scapular region. This case has been reported due to its extreme rarity and also highlights the need for including CD in the differential diagnosis of soft-tissue tumors.

How to cite this article:
Rasheed A, Khan AH, Rasool M, Shafi A. Unicentric Castleman's disease located in the scapular region.J Can Res Ther 2014;10:737-738

How to cite this URL:
Rasheed A, Khan AH, Rasool M, Shafi A. Unicentric Castleman's disease located in the scapular region. J Can Res Ther [serial online] 2014 [cited 2021 Dec 1 ];10:737-738
Available from:

Full Text


Castleman's disease (CD) is regarded as a polyclonal lymphoid proliferation of unknown etiology [1] with synonyms of giant node hyperplasia, angiomatous lymphoid hamartoma, angiofollicular lymph node hyperplasia and follicular lymphoreticuloma. [2] CD may occur anywhere along the lymphatic system with the majority of the cases seen in the mediastinum (70%). [3] Extrathoracic sites have been reported in the axilla, neck, pelvis, retroperitoneum, mesentery, central nervous system, and orbit [4] with only a handful of cases reported as intramuscular. [5]

 Case Report

A 30 years female presented to our hospital with a chief complaint of a soft-tissue mass in the right scapular region from the past three months. There was no past history of neoplastic, autoimmune or infectious diseases. Physical examination revealed firm, sharply demarcated tumor of hard consistency, and fixed to the muscles in the right blade shoulder. Her blood pressure was 120/70 and a regular pulse rate of 80/min. Her hemoglobin was 11.0 g/dL and she had a total leucocyte count of 9,300/mm 3 . Complete surgical resection was performed and an intramuscular nodule having a maximum diameter of 3.5 cm was removed and examined.


On gross examination, a sharply demarcated mass lesion embedded in hemorrhagic soft-tissue with a yellow-brown cut surface and areas of calcification was seen. Sections showed a tumor consisting of lymphoid tissue with abnormal germinal centers and marked vascular proliferation penetrating into the muscle [Figure 1]. The follicle is surrounded by a broad mantle zone consisting of a concentric layering of lymphocytes resulting in an onion-skin appearance. The follicles are frequently penetrated radially by a sclerotic blood vessel resembling a "lollipop" [Figure 2]. So, the diagnosis of CD of hyaline vascular type was established. The patient had an uneventful postoperative course and is doing well after 1 year of follow-up.{Figure 1}{Figure 2}


Castleman [6] in 1954 first described this disease in a series of 13 patients and later on in 1956 eloquently bettered the definition as a "localized mediastinal lymph node hyperplasia resembling thymoma." The disease is more frequent in women with a median age at diagnosis in the 3 rd or 4 th decade. [7]

Flendrig [8] in 1970 distinguished two basic pathologic types and one mixed variant. Based on these features Keller et al. [9] in 1972 sub-classified the disease as hyaline-vascular (HV), plasma cell (PC) and HVPC types or mixed type. CD can be clinically classified into unicentric and a multicentric disease with the former occurring in about 90% of cases, majority being of the HV type. The localized form usually has a benign course and clinical abnormalities frequently resolve after excision of the affected lymph nodes. [8] Multicentric type is usually of the PC variant and patients present with a systemic illness with a potential for developing into malignancy [10] of the non-Hodgkin's lymphoma in the absence of human immunodeficiency virus. In the localized form of plasma-cell type Hodgkin's lymphoma may occur.

Prevalence is estimated to be less than 1/100,000. [11] It has been postulated that the disease represents a reaction to chronic viral antigenic stimulation most probably to interleukin-6 in the disease associated with systemic manifestations. [12] Human herpes virus 8 HHV-8 has been implicated as the probable virus capable of causing disease. [13]

Only 10 cases of intramuscular, CD have been reported with a female predominance occurring between the age of 14 years and 48 years. [5] Most of these cases developed in the shoulder girdle. Our case conforms to the age, gender, and site as seen in the other case reports. It still remains to be elucidated whether these cases of CD presenting intramuscularly developed actually from skeletal muscle or from ectopic lymphatic tissue. [14]


CD, a rare disease and further rarer with an intramuscular origin should be kept in mind while evaluating a soft-tissue mass in the shoulder region. The exact etiology further remains to be elucidated in future studies.


1Slotwiner A, Garwacki CP, Moll S. Castleman's disease. Am J Hematol 2003;73:64-5.
2Olscamp G, Weisbrod G, Sanders D, Delarue N, Mustard R. Castleman disease: Unusual manifestations of an unusual disorder. Radiology 1980;135:43-8.
3Bowne WB, Lewis JJ, Filippa DA, Niesvizky R, Brooks AD, Burt ME, et al. The management of unicentric and multicentric Castleman's disease: A report of 16 cases and a review of the literature. Cancer 1999;85:706-17.
4Snead MP, James JN, Snead DR, Robson DK, Rizk SN. Orbital lymphomas and Castleman's disease. Eye (Lond) 1993;7:84-8.
5Schaefer IM, Günnel H, Schweyer S, Korenkov M. Unicentric castleman's disease located in the lower extremity: A case report. BMC Cancer 2011;11:352.
6Castleman B, Towne VW. Case records of the massachusetts general hospital: case 40011. N Engl J Med 1954;250:26-30.
7Tey HL, Tang MB. A case of paraneoplastic pemphigus associated with Castleman's disease presenting as erosive lichen planus. Clin Exp Dermatol 2009;34:e754-6.
8Flendrig JA. Benign giant lymphoma: Clinicopathologic correlation study. In: Clark RL, Cumly RW, editors. The Year Book of Cancer. Chicago: Yearbook Medical Publishers; 1970. p. 296-9.
9Keller AR, Hochholzer L, Castleman B: Hyaline-vascular and plasma-cell types of giant lymph node hyperplasia of the mediastinum and other locations.Cancer. 1972;29:670-683.
10Peterson BA, Frizzera G. Multicentric Castleman's disease. Semin Oncol 1993;20:636-47.
11Dégot T, Métivier A-, Casnedi S, Chenard M-, Kessler R. Thoracic manifestations of Castleman's disease. Rev Pneumol Clin 2009;65:101-7.
12Leger-Ravet MB, Peuchmaur M, Devergne O, Audouin J, Raphael M, Van Damme J, et al. Interleukin-6 gene expression in Castleman's disease. Blood 1991;78:2923-30.
13Amin HM, Medeiros LJ, Manning JT, Jones D. Dissolution of the lymphoid follicle is a feature of the HHV8+ variant of plasma cell Castleman's disease. Am J Surg Pathol 2003;27:91-100.
14Hakozaki M, Tajino T, Yamada H, Kikuchi S, Hashimoto Y, Konno S. Intramuscular Castleman's disease of the deltoid: A case report and review of the literature. Skeletal Radiol 2010;39:715-9.