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Year : 2014  |  Volume : 7  |  Issue : 3  |  Page : 346-348  

Unicentric Castleman disease at an unusual site

Department of Pathology, Padmashree Dr. D. Y. Patil Medical College, Hospital and Research Center, Dr. D. Y. Patil Vidyapeeth, Pimpri, Pune, Maharashtra, India

Date of Web Publication18-Mar-2014

Correspondence Address:
Sunita Bamanikar
5 and 6, Siddharth Estates, North Avenue, Kalyani Nagar, Pune - 411 006, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0975-2870.128979

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We report a rare case of unicentric Castleman disease (UCD) in a 56-year-old lady, presenting as a soft-tissue mass in an unusual site - mid-dorsal aspect of the arm. She was otherwise asymptomatic. Excision biopsy confirmed the diagnosis. The probable etiological factors, clinical and morphological types, and histological diagnosis are discussed.

Keywords: Arm, plasma cell variant, unicentric Castleman disease

How to cite this article:
Bamanikar S, Chaudhari U, Panicker N, Buch A. Unicentric Castleman disease at an unusual site. Med J DY Patil Univ 2014;7:346-8

How to cite this URL:
Bamanikar S, Chaudhari U, Panicker N, Buch A. Unicentric Castleman disease at an unusual site. Med J DY Patil Univ [serial online] 2014 [cited 2021 Dec 3];7:346-8. Available from:

  Introduction Top

Castleman disease (CD) is a localized or systemic lymphoproliferative disorder of uncertain etiology. [1]

Depending on the extent of involvement, it may be classified as unicentric Castleman disease (UCD) or multicentric Castleman disease (MCD). Other clinical and histomorphologic variants described are hyaline-vascular variant (HVV), plasma cell variant (PCV), human herpesvirus-8 (HHV-8) associated CD, and MCD not otherwise specified. Because of its varied clinical presentations, it may mimic other benign and malignant entities in various locations. [2] Asymptomatic localized CD-PCV presenting as a soft-tissue mass in the extremities is rare, and hence, one such case is reported here.

  Case Report Top

A 56-year-old lady presented with a painful swelling over the mid-dorsal aspect of left upper arm since 5 months. There was no history of fever, night sweats, weight loss, joint pain, gastrointestinal abnormalities, or trauma. She was afebrile with absence of pallor, edema, lymphadenopathy, or clubbing. On examination, there was a solitary swelling that was 3 × 2 cm, well defined, firm in consistency, and freely mobile with normal overlying skin. Clinical diagnosis was that of a lipoma and fine needle aspiration cytology (FNAC) was suggested. Investigations revealed hematological parameters to be within normal range. Biochemical tests for liver function, blood urea, and serum creatinine were within normal limits. Ultrasonography showed a well-defined, 2.4 × 1.4 cm, hypoechoic oval lesion of possible inflammatory origin. FNAC showed cellular smears with a polymorphous population of lymphoid cells in different stages of maturation, along with plasma cells, few polymorphs, and eosinophils. The features were suggestive of reactive lymphadenitis. Subsequently, the swelling was excised and submitted for histopathologic examination. We received a single capsulated nodule that was firm in consistency and measuring 2.8 × 2 × 0.6 cm. Cut surface was solid gray-white with no hemorrhage or necrosis. Histological examination revealed a thinly capsulated nodule with numerous hyperplastic lymphoid follicles of varying sizes and prominent germinal centers [Figure 1] and [Figure 2]. The parafollicular and vascular interfollicular areas showed presence of sheets of plasma cells, with Russell bodies along with an admixture of eosinophils [Figure 3]. Considering the characteristic pathological features, a diagnosis of solitary CD-plasma cell type was made. The subsequent postoperative course was uneventful.
Figure 1: Photomicrograph showing hyperplastic follicles (arrows) of varying sizes (H&E, ×40)

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Figure 2: Photomicrograph showing hyperplastic follicles with prominent germinal centers (arrows) (H&E, ×100)

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Figure 3: Photomicrograph showing sheets of plasma cells (arrows) in the interfollicular areas (H&E, ×400)

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  Discussion Top

CD was first described by Dr. Benjamin Castleman in the year 1956, when he studied a group of patients with large thymoma-like masses in the anterior mediastinum. He described the classic pathologic features of hypervascular lymph nodes with hyalinization of vessels, which form concentric arrangements resembling Hassall's corpuscles in the thymus. [1],[2]

Pathogenesis of the disease is still an enigma. An abnormal production of a B cell growth factor, such as interleukin (IL)-6, leading to lymphoproliferation and plasma cell differentiation has been implicated. [3] This theory is supported by a case reported by Beck et al., in which they had successfully treated a patient of CD with anti-IL-6 and steroids followed by resection of the tumor, resulting in complete clinical remission of the disease. [4]

Clinically, CD is divided into two types, UCD and MCD, based on the extent of local lymph node involvement. Extranodal CD has been reported to occur commonly in the mediastinum. [1],[2] The recent classification includes four types: the classic HVV, the less common PCV, the MCD, and CD associated with HHV-8. [5]

Over 90% of the cases are HVV and remaining cases are PCV. [5],[6],[7] It presents in roughly equal numbers of young adult men and women. The median age of presentation is in the fourth decade for unicentric and sixth decade for multicentric variant. [1],[5],[6]

The PCV is more likely to be associated with constitutional symptoms and abnormal laboratory findings. [5],[6] Multicentric PCV is characterized by lymphadenopathy, hepatosplenomegaly, and constitutional symptoms. Anemia, hypoalbuminemia, and hypergammaglobulinemia are also common. [4],[7] The case presented here was the rarer UCD of PCV occurring in the mid-dorsal aspect of the arm with no systemic symptoms. The clinical presentation of the disease is highly diverse and can mimic many benign (reactive lymphadenopathy, tuberculous lymphadenitis, lipoma, meningioma) and malignant disorders (lymphoreticular malignancies, sarcoma). [8],[9],[10],[11] Another case reported of unicentric CD was of a 28-year-old female who presented with a right axillary swelling that was clinically suspected to be lipoma or neurofibroma but turned out to be solitary form of CD-HVV on histopathologic examination following excision. [12]

CD manifests as hyperenhancing lymph nodal mass on imaging and can be considered in the differential diagnosis of lymphoma, metastatic adenopathy, infectious and/or inflammatory diseases that result in adenopathy. [2] Symptoms of Hodgkin's disease and lymphoma can mimic those of CD and can be evaluated and differentiated by a histopathologic examination. [9]

FNAC is often not conclusive; most FNACs of CD in literature are reported as reactive lymphadenitis. [13]

Extranodal CD is rare, [9] and the condition presenting as an isolated soft-tissue mass in the extremities is rarer. Our case presented with a subcutaneous soft-tissue mass in the arm, posing a clinical diagnostic challenge. A case of UCD-HVV presenting as subcutaneous soft-tissue mass has been reported. [14] Histologically, the CD-HVV shows increased number of scattered large follicles, many of which are with hyalinization and marked vascular proliferation of the germinal centers. Often, capillaries penetrating follicles give a "lollipop" appearance. There is a tight concentric layering of lymphocytes at the periphery of the follicle, resulting in an "onion-skin" or "stadium seating" appearance. The interfollicular area shows marked proliferation of post-capillary venules and presence of plasma cells, eosinophils, immunoblasts, and monocytes. Sinuses are characteristically absent. The CD-PCV is characterized by a diffuse plasma cell proliferation in the interfollicular tissue, sometimes accompanied by numerous Russell bodies, as was present in our case. The hyaline-vascular changes are inconspicuous or absent. [5],[7]

There is no standard treatment for patients with MCD; however, UCD has been treated effectively with surgical excision and carries better prognosis compared to MCD. [5] Widespread CD follows an aggressive, often fatal clinical course, and the prognosis is poor, mostly caused by infectious complications or the development of malignancies. [9],[14] Such patients often need multimodality therapy such as steroids, immunosuppressants (antiproliferative agents, including anti-IL-6 receptor therapies, and anti-CD20 antibody therapy), antineoplastic agents, and surgery. [4]

  Conclusion Top

As in most reported cases in literature, CD was not suspected clinically at all and was diagnosed subsequently only on histopathology. The unique feature of our case lies in its localization in subcutaneous tissue on the arm, presenting as PCV of CD. Though CD is rare, we highlight the importance of this entity to the clinicians that it should be kept in mind while investigating patients with solitary swelling or multiple lymph node swellings. We also conclude that it is difficult to diagnose CD on FNAC; however, definitive diagnosis can be established by histopathologic examination.

  References Top

1.Castleman B, Iverson L, Menendez VP. Localized mediastinal lymph node hyperplasia resembling thymoma. Cancer 1956;9:822-30.  Back to cited text no. 1
2.Bonekamp D, Horton KM, Hruban RH, Fishman EK. Castleman disease: The great mimic. Radiographics 2011;31:1793-807.  Back to cited text no. 2
3.Palestro G, Turrini F, Pagano M, Chiusa L. Castleman's disease. Adv Clin Path 1999;3:11-22.  Back to cited text no. 3
4.Beck JT, Hsu SM, Wijdenes J, Klein B, Hayden K, Jagannath S, et al. Brief report: Alleviation of systemic manifestations of cattleman disease by monoclonal anti-interleukin-6 antibody. N Engl J Med 1994;330:602-5.  Back to cited text no. 4
5.Cronin DM, Warnke RA. Castleman disease: An update on classification and the spectrum of associated lesions. Adv Anat Pathol 2009;16:236-46.  Back to cited text no. 5
6.Keller AR, Hochholzer L, Castleman B. Hyaline-vascular and plasma-cell types of giant lymph node hyperplasia of the mediastinum and other locations. Cancer 1972;29:670-83.  Back to cited text no. 6
7.Rosai J. Lymph nodes. In: Rosai J, editor. Rosai and Ackerman′s Surgical Pathology. 10 th ed. St Louis: Mosby; 2011. p. 1796-9.  Back to cited text no. 7
8.Demellawy DE, Herath C, Truong F, Nasr A, Alowami S. Localized early mesenteric Castleman disease presenting as recurrent intestinal obstruction: A case report. Diagn Pathol 2009;4:42.  Back to cited text no. 8
9.Masood I, Dutta V, Singh S, Parveen S. Castleman Disease: A case report and brief review of the literature. Research and reviews: Journal of Medical and Health Sciences 2013;2:1-3.  Back to cited text no. 9
10.K V R, D'Souza CR, Saldanha E, Dantis K, Rai P. Unicentric mesenteric Castleman disease-diagnostic quandary - A case report. J Clin Diagn Res 2013;7:573-5   Back to cited text no. 10
11.Hashimoto H, Iida J, Hironaka Y, Sakaki T. Intracranial Castleman's disease of solitary form. Case report. J Neurosurg 1999;90:563-6.  Back to cited text no. 11
12.Joshi SR, Tekwani T. Solitary Castleman disease-At an unusual site (Axilla) - A case report. Indian J Basic Appl Med Res 2013;2:582-4.   Back to cited text no. 12
13.Ayyagari S, Namala V. Cytologic picture of Castleman disease: A report of two cases. J Cytol 2010;27:152-4.  Back to cited text no. 13
14.Naghashpour M, Cualing HD, Szabunio M, Bui MM. Hyaline-vascular Castleman disease: A rare cause of solitary subcutaneous soft tissue mass. Am J Dermatopathol 2010;32:293-7.  Back to cited text no. 14


  [Figure 1], [Figure 2], [Figure 3]


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