Transoral robotic surgery tongue base debulking in Castleman’s disease| ACTA Otorhinolaryngologica Italica

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Dear Editor,

Transoral robotic surgery has undergone improvements in recent years and increased diffusion can implicate changes in the surgical approach of many conditions involving Waldeyer’s ring structures, even the less common ones. Castleman’s disease is a rare lymphoproliferative disorder involving lymphatic structures with the possibility of unicentric or multicentric involvement. It usually arises as a solitary mass, commonly found in the mediastinum (60%), neck (14%), retroperitoneum (11%), or axilla (4%) ¹. Its aetiology is unknown, but a role of viral infection is hypothesised (Herpesviridae, HHV8) involving the B-cell pool and the lymphovascular compartment of lymph nodes ². Histological classification differentiates hyaline vascular versus plasma cell disease according to the pattern of destruction of normal lymph node morphology, with the possibility of a mixed type including both ³. Surgical treatment is the main therapeutic option, mostly for the unicentric type ⁴. The neck is the second most frequent location, mainly in lymph nodes ^1,3, but with the possibility of lymphoreticular structures involvement, such as Waldeyer’s ring.

We report a case of a 25-year-old male, with no relevant comorbidities, who had been submitted to tonsillectomy in infancy and at 20 years of age to right axillary lymph node removal, with consequent histologic diagnosis of unifocal Castleman’s disease, hyaline vascular type. Total body computed tomography (CT) showed no other involvement of lymphatic structures. Three months before our treatment, the patient reported acute dysphagia, with cough, fever (38°C), and no blood count alterations, for about 5 days prior to endoscopy with detection of tongue base hyperplasia and symmetric ulcerative aspects on both sides (Fig. 1A). Antibiotic and cortisone therapy were administered with almost complete regression of symptoms after about 10 days. The patient was then submitted to magnetic resonance imaging (MRI, Fig. 1B) which showed symmetric tongue base hypertrophy with low and homogeneous contrast enhancement, bilateral second and third neck levels lymph nodes with augmented diameters up to 2.5 cm. The persistence of some symptoms such as sense of constriction, slight dysphagia, and snoring with sleep apnoea, led the patient to our centre, where he was submitted to endoscopy with detection of nasopharynx and tongue base hypertrophy, with symmetric aspects and no detection of mucosal irregularities or ulcerations. He gave informed consent concerning the proposed procedure of transoral robotic surgery (TORS) with tongue base hypertrophy debulking under general anaesthesia with a Da Vinci robot (Ab medica s.p.a., Rome, Italy). The surgical procedure was performed after one month using a diamond-shaped excision, on both sides about 15 mm far from the midline and 15 mm deep in the genioglossus muscle. In this way, a 3 cm wide space was achieved at the tongue base level (Fig. 1C). In the following two post-surgical hospitalisation days he received intravenous antibiotics, corticosteroids, and an analgesic, with progressive reduction of pain and dysphagia; oral therapy continued for 5 days after discharge.

His symptoms completely disappeared within two weeks from surgery and the endoscopic aspect at 3 months showed restoration of tongue base volume with almost complete mucosal re-epithelialisation (Fig. 1D). Histologic examination confirmed Castleman’s hyaline vascular type disease.

Unicentric Castleman’s disease responds very well to surgery as the sole treatment modality ⁴. The surgical approach should aim to resect the mainly involved lymph node with free resection margins, or, if a group of lymph nodes is involved, perform systematic lymphadenectomy. Failure to resect the primary involved lymph node is the only significant predictor of fatal outcome ⁴.

The goal of the surgery is complete excision of the involved lymph node tissue, with free resection margins. In most patients with unicentric disease, such a surgical approach is possible. Surgery without other multimodal approaches should be considered the gold standard for the treatment of Castelman’s disease ⁴. The surgical approach allows diagnosis to be made through biopsy and the pathology to be treated.

Visceral localisation of the dominant disease focus does not preclude the success of the surgical approach; in this case, the approach will be chosen based on the relevant techniques and specific operable conditions.

In multicentric disease, surgery assumes a noncurative role, but subordinate to biopsy sampling of tissue to make a diagnosis and debulking of dominant disease foci in the presence of specific organ-related indications, such as vascular or airway compromise, massive organomegaly, or intestinal obstruction ⁵. Future controlled studies will clarify the potential role of surgery in a multimodal therapeutic approach.

Over time, many different techniques and technologies have been developed to treat the tongue base. Approaches without tissue resection include submucosal radiofrequency, advancement of the genioglossus muscle, tongue suspension/stabilisation and hyoid bone suspension, and approaches with tissue resection, partial glossectomies, such as minimally invasive submucosal lingual excision, various approaches for lingual tonsillectomy ⁶ and submucosal linguoplasty ⁷.

In 2009, the Food and Drug Administration approved the use of the Da Vinci robotic-assisted surgical system (Intuitive Surgical, Sunnyvale, CA) for resection of malignant lesions of the oropharynx. In subsequent years, the use of TORS has grown, and Vicini was the first to use this system for tongue resection in obstructive sleep apnoea in 2010 ⁸. TORS technology offers the advantage of frontal visualisation and manipulation of the base of the tongue, allowing safe and minimally-invasive resection of disease. The resection techniques differ considering the type of disease (neoplastic versus benign) and the goals of surgery (free-margin radical excision versus functional debulking). In both cases the surgeon must know the endoscopic anatomy of the tongue base. Moving from the midline to the lateral portion of the tongue base, lingual arteries are placed lateral to the genioglossus muscle, at a lateral mean distance from the midline of 11.0 ± 4.8 mm on the right side and 12.2 ± 5.9 mm on the left side. The depth ranges between 20 and 25 mm ⁹. In the present case, considering the functional goal of improving the oropharyngeal retrolingual space, the transoral robotic technique was the same used in case of sleep apnoea: this was highly conservative with preservation of both lingual arteries and hypoglossal nerves, with a median 3 cm wide space achieved at the tongue base.

Globally, systematic reviews of partial glossectomy (by TORS and non-TORS techniques) indicate the general feasibility, safety, and clinical success in appropriately selected patients. TORS has been associated with an increased risk of prolonged hospital stay, but not with an increased risk of complications ¹⁰.

In summary, Castleman’s disease is a rare disease whose therapeutic approaches vary according to the unicentricity or multicentricity of the disease. Surgery represents the gold standard of treatment in unicentric disease. The case presented by the authors is the first described of unicentric Castleman’s disease located at the level of the base of tongue treated by a surgical TORS debulking approach, with quick post-operative recovery and stable remission of symptoms.

Conflict of interest statement

Authors declares no conflict of interests, no financial relationships that could influence authors actions, no financial interest, relationships or affiliations relevant to the subject of the manuscript.

Funding

This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

Author contributions

All authors contribuited equally to the work.

Ethical consideration

Not applicable.

Figures and tables

Figure 1:A) the acute phase endoscopy with detection of tongue base hyperplasic and symmetric ulcerative aspect on both sides; B) pre-operative MRI (T2 propeller sagittal); C) diamond shaped excision, performed on both sides about 15 mm far from the midline and 15 mm deep in the genioglossus muscle, with a 3 cm wide space achieved at tongue base level; D) post-operative endoscopy (3 months after surgery).

References

1. Song JJ, Jung MH, Woo JS. Castleman’s disease of the head and neck. Eur Arch Otorhinolaryngol. 2006; 263:160-163. DOI
2. Schulte KM, Talat N. Castleman’s disease – A two compartment model of HHV8 infection. Nature Rev Clin Oncol. 2010; 7:533-543. DOI
3. Cronin DM, Warnke RA. Castleman disease: an update on classification and the spectrum of associated lesions. Adv Anat Pathol. 2009; 16:236-246. DOI
4. Talat N, Belgaumkar AP, Schulte KM. Surgery in Castleman’s disease – A systematic review of 404 published cases. Ann Surg. 2012; 255:677-684. DOI
5. Jeehong K, Barish P. Transoral robotic surgery (TORS) versus non-TORS tongue resection for obstructive sleep apnea. Laryngoscope. 2021; 131:E1735-E1740. DOI
6. Suh GD. Evaluation of open midline glossectomy in the multilevel surgical management of obstructive sleep apnea syndrome. Otolaryngol Head Neck Surg. 2013; 148:166-171. DOI
7. Gunawardena I, Robinson S, MacKay S. Submucosal lingualplasty for adult obstructive sleep apnea. Otolaryngol Head Neck Surg. 2013; 148:157-165. DOI
8. Vicini C, Dallan I, Canzi P. Transoral robotic tongue base resection in obstructive sleep apnoea-hypopnoea syndrome: a preliminary report. ORL J Otorhinolaryngol Relat Spec. 2010; 72:22-27. DOI
9. Dallan I, Seccia V, Faggioni L. Anatomical landmarks for transoral robotic tongue base surgery: comparison between endoscopic, external and radiological perspectives. Surg Radiol Anat. 2013; 35:3-10. DOI
10. Miller SC, Nguyen SA, Ong AA. Transoral robotic base of tongue reduction for obstructive sleep apnea: a systematic review and meta-analysis. Laryngoscope. 2017; 127:258-265. DOI