Castleman disease presenting in the neck: Report of a case and review of the literature




Abstract


Castleman disease is a rare lymphoproliferative disorder with two primary subtypes that vary in presentation and course. Unicentric Castleman disease (UCD) presents as a solitary mass, most commonly in the mediastinum, and rarely in the head and neck. In contrast to multicentric Castleman disease (MCD), which features peripheral lymphadenopathy and numerous systemic symptoms, UCD is not typically associated with generalized symptoms. Here, we present an unusual case of UCD presenting as a slowly expanding, isolated neck mass in a 29-year-old woman. This case demonstrates the distinguishing clinical, radiologic, and histologic findings unique to UCD, which is often misdiagnosed as lymphoma or other malignant process. These findings stand in contrast to those observed in MCD patients, and hence, offer insight into the practical aspects of diagnosis and management of Castleman disease in the head and neck.



Introduction


Castleman disease (CD) was originally identified by Benjamin Castleman, who described a cohort of patients with solitary hyperplastic mediastinal lymph nodes which demonstrated small, hyalinized follicles and interfollicular vascular proliferation on histopathology. Years later, Castleman and colleagues distinguished this hyaline vascular type of CD from other variants of this disorder, namely the plasma cell variant and the much rarer mixed variant . The patients in these seminal studies had limited localized disease, known as unicentric Castleman disease (UCD); however, a multicentric form of CD (MCD), characterized by multiple systemic symptoms including hepatosplenomegaly, recurrent fevers, night sweats, and lymphadenopathy, was later described.


Although CD can develop in peripheral lymph nodes, it classically arises as a solitary nodal mass within the mediastinal compartments. The disease typically manifests in thoracic lymph nodes (70% of cases), but cases have been described in the pelvis, abdomen, retroperitoneum, skeletal muscle, and head and neck . UCD most often presents as a painless mediastinal mass (median size of 5–9 cm) and can be accompanied by complaints of cough, dyspnea, or constitutional symptoms. In this report, we describe an unusual case of UCD presenting in the neck. This case highlights many of the clinical features distinguishing UCD from MCD, and accordingly, serves as an instructive example of how UCD may be properly diagnosed and treated. We furthermore review the literature on both UCD and MCD and provide clinicians additional insight into the workup and management of these two unusual diagnoses.





Case report


A 29-year-old woman presented to an outside institution with a large, painless, slowly-expanding, right-sided neck mass which had developed over several months. She otherwise had few symptoms, denying pain, temperature intolerance, and weight loss, but reported mild fatigue, patchy alopecia which she attributed to stress, and intermittent night sweats, less than once a week over the past few months. An ultrasound-guided fine needle aspiration (FNA) and core needle biopsy revealed normal lymphoid tissue without evidence of malignancy. She was subsequently referred to our institution for further care. Her other medical conditions included asthma, gastroesophageal reflux, stress-induced headaches, borderline hypertension, and a history of a ureteral stone, ruptured ovarian cysts, and benign thyroid cyst. She denied smoking, alcohol consumption, and illicit drug use.


Physical examination revealed a mobile, non-tender 3 cm right Level III lymph node without overlying skin changes. Fiberoptic nasopharyngoscopy was unremarkable. Subsequent magnetic resonance imaging revealed a 1.4 × 2.1 × 3.9 cm lymph node in the right neck with no other adenopathy or masses ( Figs. 1 and 2 ), and accordingly the patient underwent an excisional lymph node biopsy for definitive diagnosis. Histopathologic examination revealed a lymph node with overall preserved architecture, containing numerous follicles of varying sizes with markedly atrophic and often vascularized germinal centers ( Fig. 3 ). The germinal centers often had sparse numbers of lymphocytes and some follicles contained two or more small germinal centers. The mantle zones were expanded, with a concentric “onion skin” arrangement of lymphocytes ( Fig. 4 ). Together, these findings were consistent with a diagnosis of Unicentric Castleman disease, hyaline vascular type.




Fig. 1


Axial post-contrast T1-weighted MR image demonstrating a hyperintense 1.4 × 2.1 × 3.9 cm lymph node in the right neck, with no evidence of other lymphadenopathy or masses.



Fig. 2


Coronal STIR sequence MR image revealing a heterogenous and hyperintense enlarged lymph node in the right neck.



Fig. 3


Histopathologic analysis of the excised node showed lymphoid tissue with overall preserved architecture. There were numerous follicles of varying sizes with markedly atrophic and often vascularized germinal centers and mildly hypervascular interfollicular tissue (H&E; 10 ×).



Fig. 4


Mantle zones demonstrating an expanded and concentric “onion skin” arrangement of lymphocytes. Germinal centers were populated with sparse numbers of lymphocytes, while some follicles contained two or more small germinal centers (H&E, 100 ×).


Complete resection was confirmed by computed tomography and the patient’s constitutional symptoms resolved soon after excision of the involved node. She was subsequently referred to radiation oncology, with a plan for local radiation therapy should her disease recur. She currently remains stable with no signs or symptoms of recurrent disease for over one year.





Case report


A 29-year-old woman presented to an outside institution with a large, painless, slowly-expanding, right-sided neck mass which had developed over several months. She otherwise had few symptoms, denying pain, temperature intolerance, and weight loss, but reported mild fatigue, patchy alopecia which she attributed to stress, and intermittent night sweats, less than once a week over the past few months. An ultrasound-guided fine needle aspiration (FNA) and core needle biopsy revealed normal lymphoid tissue without evidence of malignancy. She was subsequently referred to our institution for further care. Her other medical conditions included asthma, gastroesophageal reflux, stress-induced headaches, borderline hypertension, and a history of a ureteral stone, ruptured ovarian cysts, and benign thyroid cyst. She denied smoking, alcohol consumption, and illicit drug use.


Physical examination revealed a mobile, non-tender 3 cm right Level III lymph node without overlying skin changes. Fiberoptic nasopharyngoscopy was unremarkable. Subsequent magnetic resonance imaging revealed a 1.4 × 2.1 × 3.9 cm lymph node in the right neck with no other adenopathy or masses ( Figs. 1 and 2 ), and accordingly the patient underwent an excisional lymph node biopsy for definitive diagnosis. Histopathologic examination revealed a lymph node with overall preserved architecture, containing numerous follicles of varying sizes with markedly atrophic and often vascularized germinal centers ( Fig. 3 ). The germinal centers often had sparse numbers of lymphocytes and some follicles contained two or more small germinal centers. The mantle zones were expanded, with a concentric “onion skin” arrangement of lymphocytes ( Fig. 4 ). Together, these findings were consistent with a diagnosis of Unicentric Castleman disease, hyaline vascular type.




Fig. 1


Axial post-contrast T1-weighted MR image demonstrating a hyperintense 1.4 × 2.1 × 3.9 cm lymph node in the right neck, with no evidence of other lymphadenopathy or masses.



Fig. 2


Coronal STIR sequence MR image revealing a heterogenous and hyperintense enlarged lymph node in the right neck.

Aug 25, 2017 | Posted by in OTOLARYNGOLOGY | Comments Off on Castleman disease presenting in the neck: Report of a case and review of the literature

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