Multicentric Castleman Disease of the Supraglottis: A Surgeons' Dilemma

Abstract

Introduction: An abnormal mass in the head and neck involving the supraglottic and cervical region offers a wide range of differential diagnoses. The pathology is either benign or malignant in nature. Castleman disease (CD) is an uncommon lymphoproliferative disorder characterised by hypervascular lymphoid hyperplasia and is classified into unicentric or multicentric disease. Histopathologically it is divided into hyaline vascular (HV), plasma cell (PC), and mixed cellularity variants. The multicentric disease is linked with PC and has the propensity to progress to lymphoma or Kaposi Sarcoma.

Case report: We report a case of a 45-year-old gentleman who presented with a painless anterior neck swelling and left supraglottic mass for six months. Computed tomography (CT) contrast imaging demonstrated a homogenous enhancing lesion at the left supraglottic and the midline of the anterior neck with erosive changes of the thyroid cartilage. A surgical resection of the anterior neck mass was performed. The diagnosis of Castleman disease plasma cell variant was made by histopathologic evaluation. The patient remained well post-resection.

Conclusion: Supraglottic multicentric Castleman disease is the least expected diagnosis in this case. Unicentric disease is treated with surgery. However, limited studies are available in determining the effectiveness of surgery in multicentric diseases. The plasma cell variant requires a multidisciplinary and multimodal approach due to an inclination towards malignancy. Research is needed to determine the role of surgery in multicentric disease and to develop optimum guidelines for managing cases. To date, there is unsubstantial literature describing supraglottic multicentric disease.

Keywords: Castleman disease; Lymphoproliferative disorders; Neck; Pharyngectomy; Plasma cells.

Fig 1

Anterior midline neck swelling measuring 3cm x 3 cm

Fig 2

Computed tomographic scan contrast of the neck showing homogenously enhancing lesion at left supraglottic region measuring 2.3 x 1.7 x 2.5cm (white arrows). This lesion is causing widening of the paraglottic fat with medialisation of mucosal space

Fig 3

Computed tomographic scan contrast showing homogenously enhancing lesion at midline anterior neck at thyroid cartilage level measuring 1.3 x 2.5 x 2.1cm (white arrows). No clear plane with bilateral strap muscles. Features of erosion of the thyroid cartilage

Fig 4

Direct laryngoscope view: The left epiglottis, lingual and laryngeal surfaces of epiglottis is edematous

Fig 5

Anterior neck dissection specimen removed enbloc together with the lymphatic nodes

Fig 6

Involuted follicles with hyalinised blood vessels penetration. (x10 magnification)