Mediastinal syndrome as the presenting manifestation of classical Hodgkin lymphoma, nodular sclerosis subtype, syncytial variant, in a young woman
DOI:
https://doi.org/10.70577/xmsbqz89Keywords:
Hodgkin lymphoma; mediastinum; syncytial variant; mediastinal syndrome; young adultAbstract
The syncytial variant of classical Hodgkin lymphoma, nodular sclerosis subtype, is an uncommon entity that may present as a bulky mediastinal mass, posing significant diagnostic challenges. The aim of this study was to describe a clinical case with an initial vascular mimicry and discuss its main diagnostic implications in light of the current literature. A retrospective case report was conducted based on the patient's medical records, laboratory findings, chest radiography, computed tomography, magnetic resonance imaging, and histopathological report, complemented by a narrative review of the literature on mediastinal masses in young adults. The case involved a 21-year-old woman presenting with fever, weight loss, polyarthralgia, facial and peripheral edema, cough, and mediastinal widening. The initial diagnostic approach was directed toward vascular pathology because of differential blood pressure measurements and compressive signs. Computed tomography revealed a bulky left mediastinal mass with pulmonary infiltration, pleural effusion, and pericardial effusion. Magnetic resonance imaging ruled out a primary vascular origin and demonstrated central necrosis. Thoracotomy with biopsy established the diagnosis of classical Hodgkin lymphoma, nodular sclerosis subtype, syncytial variant, grade 2. Limitations of the case included the absence of immunohistochemical analysis, and the patient died before formal staging and initiation of disease-specific treatment. In conclusion, mediastinal syndrome with vascular mimicry may delay the diagnosis of Hodgkin lymphoma. In this setting, timely acquisition of adequate tissue through an excisional or sufficiently large biopsy is essential to establish the diagnosis and facilitate prompt therapeutic intervention in atypical presentations.
Downloads
References
Ahuja, J., Strange, C. D., Agrawal, R., Erasmus, L. T., & Truong, M. T. (2023). Approach to imaging of mediastinal masses. Diagnostics, 13(20), 3171. https://doi.org/10.3390/diagnostics13203171 DOI: https://doi.org/10.3390/diagnostics13203171
Al-Antary, E., Jesudas, R., George, A., Poulik, J., & Savaşan, S. (2019). Syncytial variant nodular sclerosis classical Hodgkin lymphoma in an adolescent and review of the literature: A unique entity. Journal of Pediatric Hematology/Oncology, 41(3), e167–e170. https://doi.org/10.1097/MPH.0000000000001245 DOI: https://doi.org/10.1097/MPH.0000000000001245
Arrioja Salazar, A. S., & Velazco, C. L. E. (2018). Linfoma de Hodgkin… una “pequeña masa” mediastinal. Revista de Ecocardiografía Práctica y Otras Técnicas de Imagen Cardíaca, 1(3), 39–41. https://doi.org/10.37615/retic.v1n3a9 DOI: https://doi.org/10.37615/retic.v1n3a9
Banerjee, A., Bhuller, K., Sudhir, R., & Bajaj, A. (2021). Diagnostic dilemma of Hodgkin’s lymphoma versus tuberculosis: A case report and review of the literature. Journal of Medical Case Reports, 15(1), 351. https://doi.org/10.1186/s13256-021-02927-x DOI: https://doi.org/10.1186/s13256-021-02927-x
Bosch-Schips, J., Granai, M., Quintanilla-Martinez, L., & Fend, F. (2022). The grey zones of classic Hodgkin lymphoma. Cancers, 14(3), 742. https://doi.org/10.3390/cancers14030742 DOI: https://doi.org/10.3390/cancers14030742
Eichenauer, D. A., Aleman, B. M. P., André, M., Federico, M., Hutchings, M., Illidge, T., Casasnovas, R.-O., Engert, A., & Ladetto, M. (2018). Hodgkin lymphoma: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Annals of Oncology, 29(Suppl 4), iv19–iv29. https://doi.org/10.1093/annonc/mdy080 DOI: https://doi.org/10.1093/annonc/mdy080
Göknar, N., Çakır, E., Çakır, F. B., Kasapçopur, O., Yegen, G., Gedik, A. H., & Teber, S. (2015). A difficult case of Hodgkin lymphoma with differential diagnosis of tuberculosis and sarcoidosis. Hematology Reports, 7(2), 5644. https://doi.org/10.4081/hr.2015.5644 DOI: https://doi.org/10.4081/hr.2015.5644
Grant, C., Dunleavy, K., Eberle, F. C., Pittaluga, S., Wilson, W. H., & Jaffe, E. S. (2011). Primary mediastinal large B-cell lymphoma, classic Hodgkin lymphoma presenting in the mediastinum, and mediastinal gray zone lymphoma: What is the oncologist to do? Current Hematologic Malignancy Reports, 6(3), 157–163. https://doi.org/10.1007/s11899-011-0090-1 DOI: https://doi.org/10.1007/s11899-011-0090-1
Hayakawa, M., Nakaoka, K., Mizutani, S., & Takao, T. (1992). A case of mediastinal Hodgkin’s disease. Journal of the Japanese Practical Surgical Society, 53(2), 333–337. https://doi.org/10.3919/ringe1963.53.333 DOI: https://doi.org/10.3919/ringe1963.53.333
Levine, I., Kalisz, K., Smith, D. A., Tirumani, S. H., Ramaiya, N. H., & Alessandrino, F. (2020). Update on Hodgkin lymphoma from a radiologist’s perspective. Clinical Imaging, 65, 65–77. https://doi.org/10.1016/j.clinimag.2020.04.021 DOI: https://doi.org/10.1016/j.clinimag.2020.04.021
McCarten, K. M., Metzger, M. L., Drachtman, R. A., Pei, Q., Friedman, D. L., Schwartz, C. L., & Kelly, K. M. (2018). Significance of pleural effusion at diagnosis in pediatric Hodgkin lymphoma: A report from Children’s Oncology Group protocol AHOD0031. Pediatric Radiology, 48(12), 1736–1744. https://doi.org/10.1007/s00247-018-4197-6 DOI: https://doi.org/10.1007/s00247-018-4197-6
Moisi, M. I., Vesa, C., Roşan, L., Vlad, L., Badiu, K., & Popescu, M. I. (2019). The tumoral etiology of the superior cava vein syndrome presenting as exudative pericarditis in a young patient. Internal Medicine, 16(5), 77–84. https://doi.org/10.2478/inmed-2019-0087 DOI: https://doi.org/10.2478/inmed-2019-0087
Mura, R., Pochepnia, S., Kifjak, D., Khenkina, N., & Prosch, H. (2024). A diagnostic approach to mediastinal masses in clinical practice. BJR|Open, 7(1), tzaf009. https://doi.org/10.1093/bjro/tzaf009 DOI: https://doi.org/10.1093/bjro/tzaf009
Park, I. S., Kim, L., Han, J. Y., Kim, J. M., Chu, Y. C., & Choi, S. J. (2008). Syncytial variant of nodular sclerosis Hodgkin’s lymphoma assessed by fine needle aspiration cytology. Cytopathology, 19(6), 394–397. https://doi.org/10.1111/j.1365-2303.2007.00533.x DOI: https://doi.org/10.1111/j.1365-2303.2007.00533.x
Perry, A. M., Smith, L. B., & Bagg, A. (2021). Classic Hodgkin lymphoma—Old disease, new directions: An update on pathology, molecular features and biological prognostic markers. Acta Medica Academica, 50(1), 110. https://doi.org/10.5644/ama2006-124.329 DOI: https://doi.org/10.5644/ama2006-124.329
Piña-Oviedo, S., & Moran, C. A. (2016). Primary mediastinal classical Hodgkin lymphoma. Advances in Anatomic Pathology, 23(5), 285–309. https://doi.org/10.1097/PAP.0000000000000119 DOI: https://doi.org/10.1097/PAP.0000000000000119
Qi, S., Milgrom, S., Dabaja, B., Tsang, R., Levis, M., Ricardi, U., & Hoppe, R. T. (2020). Two distinct prognostic groups in advanced-stage Hodgkin lymphoma revealed by the presence and site of bulky disease. Blood Advances, 4(9), 2064–2072. https://doi.org/10.1182/bloodadvances.2019001265 DOI: https://doi.org/10.1182/bloodadvances.2019001265
Rizvi, I., Zaman, S., Zaidi, N., Ashraf, S. M. K., Kumar, A., Gupta, A., & Ahmad, M. (2012). Superior vena cava syndrome caused by Hodgkin’s lymphoma in an adolescent girl. BMJ Case Reports, 2012, bcr0120125487. https://doi.org/10.1136/bcr.01.2012.5487 DOI: https://doi.org/10.1136/bcr.01.2012.5487
Satou, A., Takahara, T., & Nakamura, S. (2022). An update on the pathology and molecular features of Hodgkin lymphoma. Cancers, 14(11), 2647. https://doi.org/10.3390/cancers14112647 DOI: https://doi.org/10.3390/cancers14112647
Sethi, T., Nguyen, V., Li, S., Morgan, D., Greer, J., & Reddy, N. (2017). Differences in outcome of patients with syncytial variant Hodgkin lymphoma compared with typical nodular sclerosis Hodgkin lymphoma. Therapeutic Advances in Hematology, 8(1), 13–20. https://doi.org/10.1177/2040620716676256 DOI: https://doi.org/10.1177/2040620716676256
Shahrzad, M., Le, T. S. M., Silva, M., Bankier, A. A., & Eisenberg, R. L. (2014). Anterior mediastinal masses. American Journal of Roentgenology, 203(2), W128–W138. https://doi.org/10.2214/AJR.13.11998 DOI: https://doi.org/10.2214/AJR.13.11998
Strickler, J. G., Michie, S. A., Warnke, R. A., & Dorfman, R. F. (1986). The “syncytial variant” of nodular sclerosing Hodgkin’s disease. The American Journal of Surgical Pathology, 10(7), 470–477. https://doi.org/10.1097/00000478-198607000-00004 DOI: https://doi.org/10.1097/00000478-198607000-00004
Takahara, T., Satou, A., Tsuzuki, T., & Nakamura, S. (2022). Hodgkin lymphoma: Biology and differential diagnostic problem. Diagnostics, 12(6), 1507. https://doi.org/10.3390/diagnostics12061507 DOI: https://doi.org/10.3390/diagnostics12061507
Takeda, T., Yamaguchi, Y., Urabe, N., Saitoh, H., Momiki, S., & Fuzisawa, T. (1990). Nodular sclerosis type of mediastinal Hodgkin’s disease: A case report. Journal of the Japanese Association for Chest Surgery, 4(3), 392–397. https://doi.org/10.2995/jacsurg1987.4.392 DOI: https://doi.org/10.2995/jacsurg1987.4.392
Wang, W., Zhang, Q., & Medeiros, L. J. (2021). Syncytial variant of nodular sclerosis Hodgkin lymphoma: An under-emphasized variant. Human Pathology, 117, 115–125. https://doi.org/10.1016/j.humpath.2021.05.008 DOI: https://doi.org/10.1016/j.humpath.2021.05.008
Downloads
Published
Issue
Section
License
Copyright (c) 2026 Miguel A. Landázuri Lainez, Verónica Liseth Jiménez Torres, Cristhian Andrés Velásquez Álvarez (Autor/a)
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.
