Castleman Disease Presenting with Pseudotumour Cerebri and Myasthenia Gravis: A Case Report and Literature Review

Abstract

Castleman disease (CD) is a rare lymphoproliferative disorder that may present with various autoimmune, inflammatory, or neurologic syndromes. This is a case of a 21-year-old woman who presented with signs and symptoms of pseudotumour cerebri (PTC) who subsequently developed myasthenia gravis (MG), and was incidentally found to have a large mass in the posterior mediastinum. Upon resection, the mass was classified as unicentric CD involved with follicular dendritic cell sarcoma. Following treatment with IVIG in the setting of progressive weakness and dyspnea, she has had complete symptom resolution while maintained on a low dose of pyridostigmine for the last two years. There are 13 cases of MG and five cases of optic disc edema described as PTC associated with CD in the literature, but to our knowledge, this is the sole case reported of the intersection of all three conditions in one patient. Increased serum levels of interleukin-6 and vascular endothelial growth factor may provide clues as to the association of CD with these neurologic syndromes.

Dysphagia and Dysphonia, a Pairing of Symptoms Caused by an Unusual Pair of Diseases: Castleman’s Disease and Myasthenia Gravis

“…To date, fewer than 15 published cases of concomitant CD and MG have been published with most cases representing localized hyaline vascular variant and only 3 reported multicentric. 3 A review of 14 published cases and the one presented here shows that patients have an average age of 35.2 ± 17.2 years. The cohort is 40% male.…”

“…Castleman’s disease is a rare lymphoproliferative disorder first described in 1956. 2 It is characterized by angiolymphatic hyperplasia of the lymph nodes 3 and is classified by extent of the disease as either unicentric with single lymph node involvement or multicentric with systemic involvement. Unicentric disease is usually discovered incidentally on examination or imaging.…”

“…CD is classified into three categories according to histopathologic findings: hyaline vascular, plasmocytic or mixed patterns. 3,7-9 Ninety percent of unicentric cases are of the hyaline vascular type, characterized by enlarged lymphoid follicles composed of regressed or atrophic germinal centers traversed by radiating small hyalinized vessels and mantle zones arranged ring-like or “onion-skin” alignments. In our patient the interfollicular regions of the lymph node contained a proliferation of myofibroblasts and endothelial cells producing a stroma-rich variant of CD.…”

"Cervical Castleman’s Disease Associated with Nodular Goiter: A Case Report"

“…Individuals with multicentric Castleman's disease tend to be older (50 to 70 years of age). Considerable interest is currently being focused on the role of human herpesvirus-8 in this lesion [6,7] and lies between element with the white blood cell -6 (IL-6) to increase related [8,9]. The multicentric form of the disease is quite virulent compared with the typically benign course of classic Castleman's disease.…”

A Case of Unicentric Castleman Disease with Concomitant Myasthenia Gravis and Persistent Left Superior Vena Cava

“…In a previous report, up to 37.5% of these cases were associated with myasthenic crisis after surgical resection, an association much higher than the 5.4% incidence of myasthenic crisis following surgical resection of thymic epithelial tumors [8,9]. Table 1 includes the initial cases reported by Ishikawa et al, in addition to those reported after 2013 [3,[13][14][15][16][17][18]. One case of Castleman disease complicated by both myasthenia gravis and paraneoplastic pemphigus also included a myasthenic crisis; however, the crisis occurred during a hospitalization after a mucosal biopsy and not after the surgical resection of the mass [13].…”