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Year : 2014 | Volume : 2 | Issue : 1 | Page : 59 - 61  


Case Reports
Castleman’s Disease in Children–Hyaline Vascular Type-A Rarity

Kandukuri Mahesh Kumar1, Shashi Kiran Kayetha2, Chintakindi Sravan3, V. Vijaya Sreedhar4.

1Assistant Professor of Pathology, Malla Reddy Institute Of Medical Sciences, Hyderabad

2Consultant Pathologist, Dept. Of Pathology, Omega Cancer Hospital, Hyderabad.

3Consultant Pathologist, Dept. of Pathology, Vijaya Diagnostic Centre, Hyderabad.

4Prof & HOD of Pathology, Osmania Medical College, Hyderabad.

Abstract:

Castleman’s disease is a benign lympho proliferative disorder of an unknown aetiology, occurring rarely in the paediatric population. It primarily occurs at the sites like chest, abdomen and rarely in the neck. We report two cases of Castleman’s disease in 7-year-old girl and 11 year old girl presenting with a localised mass in the left and right neck region respectively, complaints of vague clinical symptoms and local examination revealed a soft, non-tender and immobile mass in the neck. Laboratory studies were non-contributory. Ultrasonography of the head and neck helped in defining the neck mass, though the findings were non-specific. The mass was surgically excised and subjected to pathological examination. Histopathological assessment was consistent with Castleman’s Disease, hyaline-vascular type. Follow up studies for a period of 6-months showed no evidence of recurrence of the lesion.

Keywords: Castleman’s disease, Children, Neck mass, Hyaline-vascular type

Corresponding Author: Dr. Kandukuri Mahesh Kumar, Assistant Professor, Department of Pathology, Malla Reddy Institute of Medical Sciences, Suraram, Hyderabad. Email: doctormaheshgoud@gmail.com

Introduction:

Castleman’s disease is a rare, benign lympho proliferative disorder of an unknown aetiology with its first description dating back to 1954. [1] Two theories have been postulated, one suggesting the role of immune reaction to viral infections and the other implicating interleukin 6. [2, 3, 4] In children, the disease primarily occurs at the sites like chest, abdomen and very rarely in the neck. [5] It usually presents as a solitary mass when it occurs in the neck. [6] The disease is categorized as unicentric, if it remains localized to only one lymph-node group and multicentric, if it disseminates towards two or more lymph-node groups. [7] Histopathologically, Castleman’s disease is sub-classified into hyaline-vascular, plasma cell and mixed types. [3] We report two very rare cases of Castleman’s disease, hyaline-vascular type in a 7-year-old girl and 11-year-old girl presenting with a localized right neck mass.

Case Report

Case 1 - A 7-year-old girl presented to the outpatient department with a 6-months history of a painless mass in the right neck region associated with low-grade fever, excessive sweating, fatigue and unexplained weight loss. There was also a history of vomiting thrice in last two weeks. The General physical examination was normal. Systemic examination was within normal limits. On local examination, the mass on the left side of the neck measured approximately 1.5 x 1 cm and was soft, non-tender and immobile, with skin overlying the mass being normal (Figure 1 A ).

Case 2-A 11-year-old girl presented to the outpatient department with a 3-months history of a painless mass in the right neck region associated with low-grade fever, evening rise of temperature, on and off dry cough, sweating, loss of appetite and unexplained weight loss. Patient was given symptomatic treatment and antibiotics, but there was no change in the swelling size. The General physical examination was normal. Systemic examination was within normal limits. On local examination, the mass on the right side of the neck measured approximately 3 x 1 cm and was soft, non-tender and immobile, with skin overlying the mass being normal (Figure 1 B ).

Laboratory Investigations

Case 1&2: Routine laboratory investigations like complete blood picture, urine examination, were within normal ranges. Mantoux test was non-reactive.

Case 1: Radiological investigations, which included chest radiograph, was normal. Ultrasonography of the head and neck revealed a homogenous, hypo echoic mass with increased vascularity measuring approximately 1.6 cm x 0.9 cm x 1 cm in the left cervical region- possibility of necrotizing lymph node. Ultrasonography of the abdomen revealed no abnormality ruling out the multicentric, disseminated disease.

Case 2 - Radiological investigations, which included chest radiograph, was normal. Ultrasonography of the head and neck revealed a homogenous, hypo echoic mass measuring approximately 2.6 cm x 1.5 cm x 1.3 cm in the right cervical region- Possibility of tuberculous lymphadenitis. Ultrasonography of the abdomen revealed no abnormality ruling out the multicentric, disseminated disease.

Cytology:

Case 1 & 2: (Smears studied from both cases showed similar features)

Smears from fine needle aspiration of the mass lesion predominantly showed small and large lymphocytes, occasional plasma cells admixed with fibrotic strands in hemorrhagic background (Figure 2 A and B). There was no evidence of malignancy. A Cytological diagnosis of non-specific lymphadenitis was made and biopsy advised to arrive at definite opinion.

The neck masses were surgically excised and biopsy specimen sent to the department of pathology for gross examination and histopathological assessment. The post-operative period remained uneventful and 6-month follow-up period showed no evidence of the disease progression or recurrence.

Pathology Findings

Gross Examination: Case 1 - Grossly, the specimen of excision biopsy was a single lymph node measuring 1.5 cm x 1 cm x 0.8 cm. Cut section was fleshy and showed homogenous, grey, white areas with foci peripherally showing a grey brown area.

Case 2 - Grossly, the specimen of excision biopsy was a single lymph node measuring 2.5 cm x 1.5 cm x 1.2 cm. Cut section was fleshy and showed homogenous, grey white areas.

Microscopy: (Case 1 &Case 2 showed similar microscopic features) Histopathological examination revealed lymphatic tissue having numerous germinal centers with central hyalinization and marked vascularisation. (Figure 3 A, B and C) Some lymphoid follicles showed germinal centers with concentric onion skin-like layering of surrounding lymphoid cells (Figure 3 D). There were numerous proliferating blood vessels. Based on the findings the lesion was diagnosed as Castleman’s disease, hyaline vascular type.

Discussion:

Benjamin Castleman first described Castleman’s disease in 1954 as a large mediastinal mass resembling thymoma. [8] It is a benign lympho proliferative disease with various names like giant lymphoid hyperplasia, angiomatous lymphoid hamartoma, angiofollicular lymphoid hyperplasia, or giant benign lymphoma. [7]

Little is known about the etiology of this rare lesion. One theory proposes Castleman’s disease as an immune response to viral infection, though the viral cultures remain persistently negative and demonstration of Epstein-Barr virus and Human Herpes virus type eight genome by polymerase chain reaction gives no inference whether the virus is responsible for the disease or associated with it. According to another theory, the pattern of Interleukin six genes was consistently demonstrated in Castleman’s disease and the levels of serum Interleukin 6 correlated well with clinical and biological abnormalities. [4]

The estimated prevalence rates of Castleman’s disease are 1 in 100,000 with a peak incidence in the 4th decade of life. The disease commonly presents in the adults. Although the exact prevalence rates of this disease in pediatric population remain unknown, it is still very rare. [3] Yet, the literature shows about 100 published cases of pediatric Castleman’s disease. [2] In children, the chest (33%) is the commonest site of disease, followed by the abdomen (30%), neck (23%) and axilla (7%). [6] Neck mass in children is an uncommon presentation of this disease in pediatric population. [3] To our best knowledge, there are only 31 reported cases of Castleman’s disease presenting with neck mass.

Clinically, a child with Castleman’s disease usually remains asymptomatic or may present with non-specific symptoms with a neck mass, posing a diagnostic dilemma. Often, laboratory tests are normal. Ultrasonography, Computed tomography and magnetic resonance imaging scan helps in delineating the lesion and extent of the disease. Fine needle aspiration of the lesion has its own limitations. Histopathological examination of the biopsy from the lesion helps in making a definitive diagnosis.

Treatment of the unicentric, localized disease involves surgical resection of the affected lymph node. Multicentric, disseminated disease needs medical treatment with steroids alone or in combination with agents like methotrexate, intravenous immunoglobulin or interferon. Plasmapheresis offers some success. [1]

Conclusion:

Castleman’s disease as a benign lympho proliferative disease is a rare diagnosis in the pediatric population and the disease presenting as a neck mass is even rarer. Lesions in the pediatric age group present with a diagnostic dilemma and a high index of suspicion is important for its diagnosis, since clinical manifestations are often vague and laboratory findings often non-contributory. Radiological investigations help in delineating the lesion but are inconclusive. Histopathology remains the main tool for the definitive diagnosis of the Castleman’s disease supported by clinical details and follow-up.

References:

  1. Buesing K, Perry D, Reyes C, Abdessalam S. Castleman disease: surgical cure in pediatric patients. J Pediatr Surg. 2009 Jan; 44 (1): e5-8.
  2. Farruggia P, Trizzino A, Scibetta N, Cecchetto G, Guerrieri P, D'Amore ES et al. Castleman's disease in childhood: report of three cases and review of the literature. Ital J Pediatr. 2011 Oct 20; 37:50.
  3. Zawawi F, Varshney R, Haegert DG, Daniel SJ. Castleman's Disease: A rare finding in a pediatric neck. Int J Pediatr Otorhinolaryngol. 2014 Feb; 78(2):370-2.
  4. Parez N, Bader-Meunier B, Roy CC, Dommergues JP. Paediatric Castleman disease: report of seven cases and review of the literature. Eur J Pediatr. 1999 Aug; 158(8): 631-7.
  5. Tan TY, Pang KP, Goh HK, Teo EL, Abhilash B, Walford N. Castleman's disease of the neck: a description of four cases on contrast-enhanced CT. Br J Radiol. 2004 Mar; 77 (915): 253-6.
  6. Zhong LP, Chen GF, Zhao SF. Cervical Castleman disease in children. Br J Oral Maxillofac Surg. 2004;42(1): 69-71.
  7. Bouguila J, Lahmer I, Abdelkefi M, Affissath A, Trimeche M, Boughammoura L. Cervical unicentric Castleman's disease in children. Eur Ann Otorhinolaryngol Head Neck Dis. 2013 Mar 5; 130: 221-223.
  8. Chen CC, Jiang RS, Chou G, Wang CP. Castleman’s disease of the neck. J Chin Med Assoc, 2007;70(12):556-8.

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Source of Support: Nil. Conflict of Interest: None.





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