Hyaline Vascular Variant of Castleman Disease of the Tonsil in an Adolescent: A case Report

Abstract

Castleman disease (CD) is a lymphoproliferative disorder classified into two categories as unicentric Castleman disease (UCD) or localized type and multicentric Castleman disease (MCD). A rare case of hyaline vascular variant of tonsil has been presented in which a 14 years old male presented with symptomatic unilateral hypertrophy of right tonsil. A right tonsillectomy was done and surgical pathology report was concluded as hyaline vascular variant of Castleman’s disease.Castleman disease (CD) is a rare lymphoproliferative disorder also called as giant lymph node hyperplasia, angiofollicular lymph node hyperplasia (AFH), angiomatous lymphoid hematoma and follicular lymphoreticuloma. The treatment of symptomatic patients with UCD is complete surgical excision (as in present case). In cases with incomplete resection, adjuvant radiotherapy can be given.

Introduction

Castleman disease (CD) is a benign lymph node hyperplasia, first described by Dr. Benjamin Castleman in 1954. [1] It is classified broadly into two categories as unicentric Castleman disease (UCD) or localized type and multicentric Castleman disease (MCD). [2].

UCD is more common form presenting as solitary mass which can involve cervical, axillary, mediastinal and retroperitoneal lymph nodes and are mostly asymptomatic, but can present with pressure symptoms due to the mass. In contrary to UCD, MCD is associated with systemic symptoms (fever, weight loss and night sweats). It is more severe than the unicentric form and has poorer prognosis.

CD is also divided into three pathological variants: hyaline vascular (HV), plasma cell (PC) and mixed. [3] HV is the most common variant whereas mixed type is very rare.

We are reporting a rare case of extranodal HVCD as unilateral massive tonsillar hypertrophy. Very few cases of HVCD with tonsillar involvement has been reported in literature so far. [4–6].

Case Presentation

A 14 year old male presented with complaints of dysphagia and choking episodes for 6 months, which were insidious in onset and progressive in nature. On oral examination a globular mass was seen arising from the right tonsil, crossing the midline and narrowing the oropharyngeal isthmus [Fig. 1]. The surface of the mass was smooth and firm on palpation. The left tonsillar fossa was normal. General physical examination and laboratory tests were normal. The patient had undergone computed tomography scan of neck which showed a well defined homogenously enhancing soft tissue lesion with lobulated margins, arising from right tonsil abutting the pharyngeal wall posteriorly and tonsil laterally [Fig. 2].

Fig. 1.

Transoral image showing single large smooth mass originating from right tonsil almost approaching left tonsillar pillars narrowing oropharyngeal isthmus

Fig. 2.

CECT neck (A-axial, B-coronal and C-saggital) showing a well defined homogenously enhancing soft tissue lesion with lobulated margins at the posterior end of soft palate originating from right tonsil and abutting the pharyngeal wall posteriorly

All possible differential diagnosis of unilateral tonsillar hypertrophy were kept in mind before any surgical intervention. A right tonsillectomy with mass excision was planned under general anesthesia. A well informed written consent was obtained from the parents and the patient underwent right tonsillectomy using and radiofrequency. The entire specimen (mass with tonsillar tissue) was sent for histopathological examination. The whole specimen measured 4.5 cm x 2.4 cm x 2.0 cm whereas the nodule measured 3.0 cm x 2.4 cm x 2.0 cm [Fig. 3].

Fig. 3.

Excised or tonsillectomy specimen with globular mass attched to tonsil measuring 4.5 cm x 2.4 cm x 2.0 cm (mass measures 3 cm x 2.4 cm x 2.0 cm)

Surgical pathology report [Fig. 4]: The cut section of the mass was soft, white to light brown and fairly homogenous. Sections from the nodule revealed enlarged tonsillar tissue covered with bland stratified squamous epithelium. The underlying tonsillar parenchymal tissue was hyperplastic and revealed multiple lymphoid follicles with prominent germinal centres, showing marked variation in size, with few of them about 4 to 5 times the size of normal lymphoid follicles. The germinal centres of fair number of lymphoid follicles showed atresia and infiltration by many small lymphocytes, which were closely intermingled with centroblasts, centrocytes and follicular dendritic cells. A fair number of lymphoid follicles in addition revealed one to few arterioles and small arteries with hyalinized walls, entering the germinal centre from mantle zone of the follicle. Some of these altered follicles also revealed several layers of concentrically arranged small lymphocytes in the mantle zone. Only occasional tingible body histiocytes were noted in the follicles. Similar blood vessels as seen in the follicles were also seen in the mantle zone and interfollicular areas. The interfollicular zones revealed sheets of mostly mature small lymphocytres admixed with few plasma cells and immunoblasts. Epithelioid cell granulomas were not seen. Conclusion: Hyperplastic tonsillar tissue showing features consistent with hyaline vascular variant of Castleman’s disease.

Fig. 4.

(A): low power scanning magnification shows variable sized lymphoid follicles throughout the lymph node parenchyma. (B): 20X-HE- thick walled blood vessels with compressed lumen within the atretic germinal centre of the follicle. (C): 10x HE-LF atretic germinal centre shows prominent branching blood vessels and infiltration of mature lymphocytes. (D): 10x- thick walled hyalinized blood vessel in the paracortex of lymph node. (E): 20x- A Lymphoid follicle showing prominent blood vessels entering it from the mantle zone. There is concentric arrangement of small lymphocytes at the periphery of the lymph node. (F): 10x- A large Lymphoid follicle showing infiltration of the native germinal centre with a fair number of small lymphocytes which are outnumbering the centroblasts and centrocytes. The expanded follicle is in the lower left of the image while the interfollicular area is towards the upper right of the image

Discussion

Castleman disease (CD) is a rare lymphoproliferative disorder also called as giant lymph node hyperplasia, angiofollicular lymph node hyperplasia (AFH), angiomatous lymphoid hematoma and follicular lymphoreticuloma. [4] It is the abnormal overgrowth of lymphoid tissue and involvement of a solid organ is rare. The overproduction of cytokine (IL-6) is the etiological factor in CD which leads to proliferation of B cells. [7].

CD has different clinical and pathogical subtypes, the manifestations and the management of which are different. Unicentric variant (UCD) usually presents with isolated lymphadenopathy, chest being the commonest site (29%) and the average size of lymph node involved is 5.5 cm. [8] Multicentric variant of CD (MCD) usually associated with human immunodeficiency virus (HIV), HHV-8 and malignancy (Kaposi sarcoma or lymphoma) [7, 9] and can present with fever, weight loss, pleural effusion, asthenia, ascites, neuropathy, skin rashes or edema. [2, 10] Few analytical studies had found the association of CD with some malignancies like Kaposi’s sarcoma, non-Hodgkin’s lymphoma, Hodgkin’s lymphoma and follicular dendritic cell sarcoma. [11–13].

Hyaline vascular Castleman disease (HVCD) accounts for 80–90% of UCD whereas rest 10–20% are classified as plasma cell Castleman disease (PCCD) [3] HVCD most commonly involves mediastinal nodes and is predominantly seen in younger individuals (median age-35 years) with equal male to female ratio. [13] In contrary, patients with MCD is of PC type and involve older individuals.

The differential diagnosis of unilateral mass arising from tonsil in an adolescent includes lymphoma and Kaposi sarcoma. [4, 14] In cases of unicentric involvement, excisional lymph node biopsy or tonsillectomy (in case of tonsillar mass) is the preferred method for diagnosis. Histopathological examination of the specimen is necessary for making the definitive diagnosis of Castleman disease (CD). The atrophic hyalinized germinal centres with broad mantle zones of small lymphocytes is the characteristic histopathological feature of HVCD. [3].

The treatment of symptomatic patients with UCD is complete surgical excision (as in present case). [3, 10] In cases with incomplete resection, adjuvant radiotherapy can be given. In MCD, chemotherapy, radiotherapy and immunotherapy (anti-IL-6 monoclonal antibody therapy) are the primary treatment modalities. [10, 16].

Learning Point

Unicentric Castleman disease (UCD) can rarely affect the tonsil and presents as unilateral tonsillar mass which can produce symptoms due to mass effect.

Funding

No funding received from any organization.

Declarations

Conflict of Interest

None.

Ethical issues

No ethical issue.