Abstract
Purpose
Angiosarcoma of the tongue is an exceedingly rare malignancy of the head and neck. Such lesions can be primary in nature or occur in a previously irradiated field. We examine a series of cases with relation to clinical presentation, diagnosis, management, and outcomes.
Materials and methods
Retrospective chart review of all patients with angiosarcoma of the tongue at a tertiary academic institution yielded a single case between 2005 and 2016. The MEDLINE database was additionally searched for all case series or reports of angiosarcoma arising in the tongue, and pertinent clinical data were extracted.
Results
The clinical presentation, disease course, and management of a patient with angiosarcoma of the tongue are presented. Institutional and literature search yielded a total of eight patients with angiosarcoma of the tongue. The most common primary sites were dorsal and lateral oral tongue. Treatment consisted of surgical resection in 63% of cases with adjuvant therapy administered in 75% of cases. Follow-up times varied per patient, but 63% had persistent or recurrent disease and 67% died of or with disease within two years of index presentation.
Conclusion
Angiosarcoma of the tongue is a rare and highly aggressive tumor, accounting for fewer than 1% of all head and neck malignancies. The mainstay of treatment is surgical resection with negative margins followed by adjuvant chemoradiation for high-risk features. Due to rarity of the disease, consensus on optimal treatment approach is lacking, and multi-center prospective studies would be helpful to set clinical guidelines.
1
Introduction
According to the World Health Organization database, head and neck cancer is the seventh most commonly occurring cancer in the world . Over 90% are squamous cell carcinoma arising from squamous epithelium which lines the oral cavity. The most common subsite is the oral cavity, which accounts for 38% of cases. Within the oral cavity, the most common subsite is the oral tongue, representing 32% of oral cavity cancers. Other histologic subtypes, including verrucous carcinoma, minor salivary gland carcinoma, lymphoma, soft tissue sarcoma, and metastatic lesions are much rarer. Neoplasms of the tongue can present challenges for diagnosis and management. Early in its course, a lingual neoplasm may mimic other benign oral cavity pathology; therefore, delayed diagnosis is common. Further, management of these lesions must take into account a high relative risk of locoregional spread, and can result in morbidity with alterations in oral and pharyngeal physiology, including speech and deglutition.
Angiosarcoma is a rare, malignant vascular tumor which is considered to be highly aggressive and associated with a poor prognosis. It is thought to account for approximately 1% of all soft tissue malignancies . The progenitor cells are thought to be endothelial in origin. Although it may occur in any type of soft tissue, cutaneous presentation prevails. Angiosarcoma in the head and neck is exceedingly rare, accounting for less than 4% of tumors . Angiosarcomas tend to arise spontaneously, but may also occur in a post-irradiated field . Other risk factors for angiosarcoma include longstanding lymphedema and exposure to chemical carcinogens, most commonly thorium dioxide (Thorotrast) .
The first reported case of primary tongue angiosarcoma was by Harter in 1927 . Since then, in the English literature, there have been only a few reports of angiosarcoma of the oral tongue . We report an additional case of a primary angiosarcoma of the base of tongue in a 57-year-old male patient who underwent surgical resection, with no evidence of recurrence at 8 months postoperatively. We additionally review the literature for other cases of this rare neoplasm, and summarize treatment approaches and outcomes.
2
Materials and methods
2.1
Study design and literature review
This study was approved by the Institutional Review Board of the University of California, Los Angeles Office of Research Administration. The medical records of a tertiary care hospital over eleven years were queried, yielding a single patient treated for primary angiosarcoma of the tongue. A comprehensive MEDLINE search was performed for the key words “angiosarcoma tongue” between 1950 and 2016. All reported cases of angiosarcoma of the tongue with relevant clinical information were examined. Specifically, information regarding age, sex, presenting signs and symptoms, tongue subsite, tumor size, presence of neck disease and distant metastases, TNM staging, treatment modality (e.g., surgery, radiation, chemotherapy), and clinical outcomes were extracted. Case reports with insufficient clinical information or not available in English were excluded.
2.2
Case report
The reported patient was a 57 year-old male pastor who presented with irritation and fullness in his mouth with eating and speaking. The pain was worse with chewing and associated with ipsilateral otalgia. When the symptoms did not resolve following conservative medical management elsewhere, diagnostic imaging was obtained, which revealed a 5 × 3 cm right base of tongue mass with infrahyoid extension ( Fig. 1 ). An ultrasound-guided fine needle aspiration (FNA) demonstrated cells compatible with high-grade angiosarcoma. His past medical history was remarkable for gastroesophageal reflux disease without any history of tobacco use or heavy alcohol abuse or previous head and neck irradiation. Physical examination revealed a 5 cm posterolateral floor of mouth and base of tongue firm submucosal mass extending below the mylohyoid, without associated cervical lymphadenopathy. Endoscopy showed a right base of tongue exophytic mass extending across midline down to vallecula. After discussing various treatment options, he elected for surgical excision and primary reconstruction. Tumor-negative excision included a mandibulotomy in conjunction with right hemi mobile tongue and three-quarters base of tongue resection. Functional rehabilitation was re-established with a microvascular free tissue transfer, using anterolateral thigh fasciocutaneous free flap, to provide enough bulk for eventual swallowing.
Surgical pathology showed a 5.2 cm high grade angiosarcoma with negative surgical margins and seven of twenty-two lymph nodes positive for metastases, positive perineural invasion but negative extracapsular spread, with strong and diffuse positivity for endothelial cell markers, CD31 and Erg9 ( Fig. 2 ). The patient underwent post-operative radiation therapy, and chose not to receive chemotherapy. He was decannulated postoperatively and his swallowing function improved with rehabilitation, allowing removal of his gastrostomy tube. He resumed his pastoral duties thereafter. He remained free of disease for approximately 7 months, until he developed a biopsy-proven 3.6 cm left liver metastasis treated with multiple cycles of doxorubicin and olaratumab. He later developed multiple painful thoracic vertebral lesions for which he underwent palliative localized radiation therapy, and died shortly thereafter.
2
Materials and methods
2.1
Study design and literature review
This study was approved by the Institutional Review Board of the University of California, Los Angeles Office of Research Administration. The medical records of a tertiary care hospital over eleven years were queried, yielding a single patient treated for primary angiosarcoma of the tongue. A comprehensive MEDLINE search was performed for the key words “angiosarcoma tongue” between 1950 and 2016. All reported cases of angiosarcoma of the tongue with relevant clinical information were examined. Specifically, information regarding age, sex, presenting signs and symptoms, tongue subsite, tumor size, presence of neck disease and distant metastases, TNM staging, treatment modality (e.g., surgery, radiation, chemotherapy), and clinical outcomes were extracted. Case reports with insufficient clinical information or not available in English were excluded.
2.2
Case report
The reported patient was a 57 year-old male pastor who presented with irritation and fullness in his mouth with eating and speaking. The pain was worse with chewing and associated with ipsilateral otalgia. When the symptoms did not resolve following conservative medical management elsewhere, diagnostic imaging was obtained, which revealed a 5 × 3 cm right base of tongue mass with infrahyoid extension ( Fig. 1 ). An ultrasound-guided fine needle aspiration (FNA) demonstrated cells compatible with high-grade angiosarcoma. His past medical history was remarkable for gastroesophageal reflux disease without any history of tobacco use or heavy alcohol abuse or previous head and neck irradiation. Physical examination revealed a 5 cm posterolateral floor of mouth and base of tongue firm submucosal mass extending below the mylohyoid, without associated cervical lymphadenopathy. Endoscopy showed a right base of tongue exophytic mass extending across midline down to vallecula. After discussing various treatment options, he elected for surgical excision and primary reconstruction. Tumor-negative excision included a mandibulotomy in conjunction with right hemi mobile tongue and three-quarters base of tongue resection. Functional rehabilitation was re-established with a microvascular free tissue transfer, using anterolateral thigh fasciocutaneous free flap, to provide enough bulk for eventual swallowing.
Surgical pathology showed a 5.2 cm high grade angiosarcoma with negative surgical margins and seven of twenty-two lymph nodes positive for metastases, positive perineural invasion but negative extracapsular spread, with strong and diffuse positivity for endothelial cell markers, CD31 and Erg9 ( Fig. 2 ). The patient underwent post-operative radiation therapy, and chose not to receive chemotherapy. He was decannulated postoperatively and his swallowing function improved with rehabilitation, allowing removal of his gastrostomy tube. He resumed his pastoral duties thereafter. He remained free of disease for approximately 7 months, until he developed a biopsy-proven 3.6 cm left liver metastasis treated with multiple cycles of doxorubicin and olaratumab. He later developed multiple painful thoracic vertebral lesions for which he underwent palliative localized radiation therapy, and died shortly thereafter.
