Title: Castleman disease presenting as a longstanding axillary and chest
wall mass: A case report
Wenqing Zhou1*, Xing Liu2*,
Aiming Qiu3*, Teng Ni4, Tiangeng
Dong5, Lei
Ding6
Wenqing Zhou, Xing Liu and Aiming Qiu contributed equally to this work
Correspondence should be addressed to Tiangeng Dong;
dong.tiangeng@zs-hospital.sh.cn and Lei Ding; dingleiokok@163.com
Abstract: Castleman disease is a rare
lymphoproliferative disorder that can present with various clinical
features. We present a case of a 30-year-old female with a progressively
enlarging mass in the left axilla and chest wall for ten years. After
diagnostic workup, including ultrasonography, MRI, and core needle
biopsy, the patient underwent surgical excision of the mass. The
diagnosis of Castleman disease was confirmed through pathological and
immunohistochemical examinations. The patient recovered well
postoperatively with a good prognosis.
Keyword: Castleman disease, axillary, chest wall
Introduction: Castleman disease, first described in 1956 by Dr. Benjamin
Castleman, is a rare lymphoproliferative disorder. This disease can
affect any lymphoid tissue in the body and present with localized or
systemic symptoms. The diagnosis is based on pathological examination,
and the disease has a varied clinical course and response to treatment[1].
Case report: The patient was a 30-year-old female with a ten-year
history of a progressively enlarging mass in the left axilla and chest
wall (Figure 1). The mass was initially small and asymptomatic but had
gradually increased in size over the years. The patient had no
significant medical history or family history of lymphoproliferative
disorders.
Diagnostic workup, including ultrasonography(Figure 2) and MRI[Figure
3A,3B], revealed a well-circumscribed, heterogeneously enhancing mass
measuring 10 x 6 x 3 cm . A core needle biopsy was performed, and
pathological and immunohistochemical examinations revealed clonal
lymphoid proliferation within fibrotic stroma (Figure 4A,4B). The
histopathological features were consistent with the hyaline vascular
variant of Castleman disease [2].
After appropriate preoperative preparation, the patient underwent
surgical excision of the mass. Intraoperative findings revealed a
well-encapsulated mass that was adherent to the chest wall muscles. The
mass was completely excised with negative margins. Postoperative
recovery was uneventful, and the patient was discharged on postoperative
day 5.
Histopathological examination of the excised mass confirmed the
diagnosis of Castleman disease. The immunohistochemical profile was
consistent with the hyaline vascular variant, which is characterized by
clonal proliferation of B-cells with follicular dendritic cell
expansion.
Discussion: Castleman disease is a rare lymphoproliferative disorder
that can present with varied clinical features[3].
The disease has two major histological subtypes: hyaline vascular and
plasma cell. The more common hyaline vascular subtype presents with
localized lymphadenopathy, while the less common plasma cell subtype is
associated with systemic symptoms and multiorgan
involvement[4].
Diagnosis of Castleman disease requires a combination of clinical,
radiological, and pathological findings. Treatment options include
surgery, radiation therapy, and chemotherapy, depending on the subtype,
stage, and extent of the disease[5]. The localized
hyaline vascular subtype generally has a good prognosis, while the
systemic plasma cell subtype has a more variable clinical course[6].
Conclusion: Castleman disease should be considered as a differential
diagnosis for longstanding masses in lymphoid tissue. Appropriate
diagnostic workup, including imaging and pathological examinations, is
crucial for accurate diagnosis and optimal management. Surgical excision
can offer a curative treatment with a good prognosis for the localized
hyaline vascular subtype [7].
Acknowledgments
This work was supported by grants
from the Science and Education Foundation of Wujiang District
(wwk202019).
References:
[1] Oksenhendler E. The Spectrum of Castleman’s Disease and its
Treatment. Hematology Am Soc Hematol Educ Program. 2016;2016(1):327-334.
[2] Chan KL, Tong J, Loong F, et al. Castleman’s disease: a report
of 18 cases from a single institution. J Clin Oncol.
1998;16(5):1988-1995.
[3] Soumerai JD, Sohani AR, Abramson JS. Diagnosis and management of
Castleman disease. Cancer Control. 2014;21(4):266-278.
[4] Dispenzieri A. Castleman disease. Hematology Am Soc Hematol Educ
Program. 2018;2018(1):324-329. doi:10.1182/asheducation-2018.1.324
[5] Liu AY, Nabel CS, Finkelman BS, et al. Idiopathic multicentric
Castleman’s disease: a systematic literature review. Lancet Haematol.
2016;3(4):e163-e175. doi:10.1016/S2352-3026(16)00015-X
[6] Talat N, Belgaumkar AP, Schulte KM. Surgery in Castleman’s
disease: a systematic review of 404 published cases. Ann Surg.
2012;255(4):677-684. doi:10.1097/SLA.0b013e31824a57e9
[7] van Rhee F, Stone K, Szmania S, Barlogie B, Singh Z. Castleman
disease in the 21st century: an update on diagnosis, assessment, and
therapy. Clin Adv Hematol Oncol. 2010;8(7):486-498