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We report the case of a 13-year-old girl who presented with a painless midline submental mass. Excision biopsy confirmed Castleman's disease of the hyaline-vascular type. This unusual condition needs to be considered in the differential diagnosis of masses arising in the neck.
Castleman's disease is a rare benign condition of unknown aetiology that can present as a localised mass or in a more aggressive multicentric form. The localised form of the disease tends to present as progressive painless slow-growing lymph-node enlargement. The majority of cases involve the mediastinum, but the head and neck is the second most common site of occurrence, where it can pose a diagnostic challenge. Diagnosis is possible only by histological examination. Complete excision is the treatment of choice and tends to be regarded as curative for the localised form of the disease.
1. Case report
A 13-year-old girl was referred to the Department of Oral and Maxillofacial Surgery by her general medical practitioner because of an unsightly midline neck mass. The mass had been present for 2 years, and was first noticed by the patient after a minor trauma to her chin. Examination revealed a 2 cm firm painless mobile mass in the midline of the submental triangle. The mass did not rise on swallowing. The patient was euthyroid and in good health.
MRI demonstrated a 2 cm well-defined lesion in the subcutaneous fat immediately posterior to the mental symphysis. Coronal images confirmed that the lesion was lying between the anterior bellies of the digastric muscle and anterior to the mylohyoid muscle. The lesion was of intermediate T1 signal intensity (Fig. 1) and elevated T2 signal intensity, with a pattern suggesting soft tissue. The MRI appearances were not consistent with either a lipoma or a ranula, and we decided to carry out an excisional biopsy of the mass under general anaesthesia. At operation, the lesion was found to be solid and adherent to the digastric and mylohyoid muscle bellies.
Figure 1Coronal T1-weighted MRI showing a midline lesion of low signal.
Histology of the specimen showed the typical features of the hyaline-vascular type of Castleman's disease (Fig. 2) . The lymph node was enlarged and contained numerous germinal centres, many of which were small and hyalinised, containing many follicular dendritic cells. The mantle zone was expanded and composed of tightly packed lymphocytes in a concentric arrangement around the germinal centres. The interfollicular area contained many small hyalinised vessels.
Figure 2Photomicrograph showing a hyalinised germinal centre and a concentric arrangement of small lymphocytes (H&E×120).
Castleman's disease was first described in 1954 as a form of localised mediastinal lymph-node hyperplasia characterised by germinal centre and capillary proliferation.
Clinically and histologically, two forms of the disease are now recognised. One is localised and usually of the hyaline-vascular type, the other is more aggressive, multicentric and always of the plasma cell type. The aetiology is uncertain, but interleukin-6 is thought to be responsible for the clinical and biological signs
—fever, weight loss, elevated C-reactive protein, hypergammaglobulinaemia and microcytic anaemia. There is no sex predilection and young adults are particularly affected; only 94 cases, which have generally been of the localised form of the disease, have been described in children.
The aetiology of the disease is still unclear; suggested explanations include a hamartomatous lymphoid origin and a hyperplastic response to infection, although serological and tissue-culture studies have been inconclusive.
When Castleman's disease occurs in the neck the lesions have been described as lateral rather than midline, with 52 out of the 63 reported cervical cases occurring in nodes under the sternocleidomastoid muscle. Other reported sites include the parotid gland (six cases), and there have been individual reports of cases in the floor of the mouth, the submandibular gland, the larynx, the palate and the parapharyngeal space.
To our knowledge, Castleman's disease has not previously been reported in the midline of the neck. The diagnosis is made by excision biopsy, and MRI may be helpful, particularly in showing the presence of stellate hypointensities on T2-weighted images, which may represent fibrosis in the lymph nodes.
These findings were not present in our case. Other diagnostic tests that may be useful when systemic symptoms are present include serum interleukin-6 assay, C-reactive protein and gammaglobulin assay. Fine-needle aspiration cytology can be considered where simple excision is not possible, but may lead to conflicting information.
The plasma cell type of the disease is usually multicentric, may be associated with the systemic symptoms mentioned and can occasionally progress to lymphoma. All patients with the plasma cell variant need full investigation, including CT of the chest.
In summary, although the majority of cervical Castleman's disease occurs laterally, this case demonstrates that Castleman's disease should be considered in the differential diagnosis of a solitary midline neck mass. The treatment for localised Castleman's disease is excision, which is regarded as curative.
References
Castleman B
Towne V.W
Case reports of the Massachusetts General Hospital.
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