|Year : 2014 | Volume
| 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
|Date of Web Publication||14-Oct-2014|
Dr. Ather Hafiz Khan
Department of Pathology, Sher-I-Kashmir Institute of Medical Sciences/Medical College, Bemina, Srinagar, Jammu and Kashmir
Source of Support: None, Conflict of Interest: None
Castleman'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.
Keywords: Castleman′s disease, intramuscular, unicentric
|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;10:737-8
| > Introduction|| |
Castleman's disease (CD) is regarded as a polyclonal lymphoid proliferation of unknown etiology  with synonyms of giant node hyperplasia, angiomatous lymphoid hamartoma, angiofollicular lymph node hyperplasia and follicular lymphoreticuloma.  CD may occur anywhere along the lymphatic system with the majority of the cases seen in the mediastinum (70%).  Extrathoracic sites have been reported in the axilla, neck, pelvis, retroperitoneum, mesentery, central nervous system, and orbit  with only a handful of cases reported as intramuscular. 
| > 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.
| > Pathology|| |
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: Low power targetoid pattern of hyaline vascular subtype invading the muscle (×100)|
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|Figure 2: Hyalinized germinal centers with onion skinning of lymphocytes and a targetoid pattern resembling a lollipop (×400)|
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| > Discussion|| |
Castleman  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. 
Flendrig  in 1970 distinguished two basic pathologic types and one mixed variant. Based on these features Keller et al.  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.  Multicentric type is usually of the PC variant and patients present with a systemic illness with a potential for developing into malignancy  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.  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.  Human herpes virus 8 HHV-8 has been implicated as the probable virus capable of causing disease. 
Only 10 cases of intramuscular, CD have been reported with a female predominance occurring between the age of 14 years and 48 years.  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. 
| > Conclusion|| |
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.
| > References|| |
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[Figure 1], [Figure 2]