Mesenchymal Tumors

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BENIGN MESENCHYMAL TUMORS Hemangiomas

Hemangiomas and hemangioendotheliomas are most commonly seen in the parotid gland and in children, where they account for up to 35% of salivary tumors (Ord 2004). These tumors are most commonly seen under the age of 1 year and may be present at birth, where they may exhibit aggressive growth. Hemangioendotheliomas are more aggressive and rapidly growing and occur in the <6 month infant, while the older children tend to present with the slower-growing cavernous lesions (Figure 11.1). In the past surgical removal was advocated, but as the majority of tumors involute over time, and because of the morbidity of surgery in infants, this has largely been abandoned in favor of medical therapy. Previous papers have indicated that vascular malformations of the parotid respond poorly to medical therapy; however, this has been disproved by Greene, Rogers, and Mulliken (2004). These authors reviewed 100 consecutive children with a 4.5 : 1 female to male ratio with 59% ulcerating during the proliferating phase and 89% involving nearby structures. Seventy of the patients were treated medically, 67 primarily with cortico-steroids and 3 with interferon. Initially 56/67 of the patients treated with steroids showed regression or stabilization, but 18 required further treatment with interferon. The overall response to steroids/alfa-2a or -2b interferon was 98%, and the authors concluded that parotid gland vascular tumors respond in the same way as hemangiomas elsewhere. Interestingly, 66% of the children required some form of reconstructive surgery during the involuted phase. In adults vascular lesions of the parotid are less common, but intramuscular hemangiomas of the masseter muscle can be a diagnostic challenge (Figure 11.2).

Vascular lesions of the submandibular gland are seen rarely and like vascular lesions elsewhere will be treated depending on their flow characteristics and the vessel(s) affected (Figure 11.3).

Lymphangiomas

Lymphangiomas may be capillary or cavernous (and associated with vascular malformations) or

Figure 11.1a. A 3-year-old boy with prominent cavernous vascular neoplasm of the parotid gland.

Figure 11.1b. Operative sequence shows the neoplasm is mobilized forward after ligating feeding vessels and is being peeled off the main trunk of the facial nerve (arrow).

Figure 11.1c. The vascular neoplasm is removed in total with the superficial lobe of the parotid gland after complete facial nerve dissection.

Figure 11.2. The magnetic resonance scan shows the vascular malformation to be located intramuscularly in the left masseter muscle deep to the parotid.

Figure 11.1b. Operative sequence shows the neoplasm is mobilized forward after ligating feeding vessels and is being peeled off the main trunk of the facial nerve (arrow).

Figure 11.2. The magnetic resonance scan shows the vascular malformation to be located intramuscularly in the left masseter muscle deep to the parotid.

Figures 11.3a, 11.3b, and 11.3c. Flow voids noted in the vascular malformation in the right submandibular region. At the time of surgery this was found to be cavernous and primarily venous.

be cystic in nature. They are common in the neck and seen more in the submandibular (37%) than parotid glands (31%) (Orvidas and Kasperbauer 2000). They may be prominent at birth as cystic hygromas and may pose a threat to the airway (Figure 11.4). In children they can increase considerably in size during upper respiratory tract infections. Some authors have documented the posterior triangle to be a more common site in the neck than the submandibular space, 54% versus 17%, respectively (Fageeh, Manoukian, and Tewfik et al. 1997). In a series of 324 pediatric patients with salivary gland masses, 89 (27.5%) were lymphangiomas compared to 192 (59.2%) being hemangiomas (Bentz et al. 2000). Many of these lesions are treated surgically, but persistence and recurrence are problematic (Orvidas and Kasperbauer 2000). Especially in the infiltrating lesions, complete excision may be impossible and debulk-ing is performed. Surgery can be combined with sclerosing injections or these can be used as a

Figure 11.4. Lymphangioma involving both the parotid and submandibular glands in an 8-month-old infant.

single modality. Sclerosing agents are most effective in macrocystic lymphangiomas, and in 54 of these cases 49% had excellent results, 35% good, and 16% poor using sclerosant injection (Emran, Dubois, and Laberge et al. 2006).

Neural Tumors

In Siefert and Oehne's (1986) review of 150 benign mesenchymal tumors of the salivary glands, 16% were neurogenic in origin distributed over the fourth to seventh decade. These were divided into neurilemmomas (neurinomas) in 12 of 27 cases, neurofibromas in 12 of 27 cases, and neurofibro-matosis in 3 of 27 cases. There was a predominance of males, 75% for neurofibromas but 65% females for neurilemmomas. Both MR and CT scan may be useful in imaging. In the parotid gland extension of the tumor in the gland and in the petrous bone is well defined by MR imaging, while CT scan shows bone erosion and relationship to the inner ear. A combination of CT and MR is recommended when surgical resection is planned (Martin et al. 1992).

Complete removal of these lesions, especially the plexiform neurofibroma, can be extremely difficult due to their infiltrating nature and often increased vascularity (Figure 11.5). Although approximately one-third of neurilemmomas occur in the head and neck (Almeyda et al. 2004), they are comparatively rare in the salivary glands published as isolated case reports. However, as they may be mistaken for a malignant parotid tumor due to facial nerve dysfunction—for example, progressive weakness, sudden facial paralysis, hemi-facial spasm and pain (Balle and Greisen 1984)—it is important to make the diagnosis to avoid inappropriate radical surgery. Regarding intra-parotid neurofibromas, a "conservative course of treatment with limited tumor excision and emphasis on retaining facial nerve function" is advocated (McGuirt, Johnson, and McGuirt 2003). Indeed, once the histologic diagnosis is made, because of the slow growth of the tumor and the unlikelihood of malignant change, conservative treatment of leaving the tumor in situ to preserve the nerve has been recommended (Fierke, Laskawi, and Kunze 2006).

Lipomas

Approximately 15-20% of lipomas occur in the head and neck region (Weiss and Goldblum 2001),

Figure 11.5a. Massive plexiform neurofibroma involving the parotid gland and orbit.

and in reviewing 125 lipomas in the oral and maxillofacial region, 30 (24%) were parotid and 17 (13.6%) were submandibular in orgin (Furlong, Fanburg-Smith, and Childers 2004). In this series there was a 3 : 1 male to female gender ratio and a mean age of 51.9 years. Histologically almost half (62/125) were classic lipomas, while 59 were spindle cell/pleomorphic, 2 were fibrolipomas, and 2 chondroid lipomas. Spindle cell lipomas comprised the majority of parotid lipomas. In a review of 167 mesenchymal salivary gland tumors, Seifert and Oehne (1986) found lipomas comprised 22.5% of 150 benign tumors and 95% were in the parotid. Again 85% occurred in males. A report of 660 parotid neoplasms found only 8 patients had lipomatous tumors (1.3%), 5 with focal lipoma and 3 with diffuse lipomatosis (Ethunandan, Vura, and Umar et al. 2006). Only one tumor of 8 was in the deep lobe, but small series of parotid lipomas in the deep lobe have been reported (Gooskens and Mann 2006).

Figure 11.5b. CT scan shows extensive soft tissue involvement.

Lipomas are comparatively rare in the oral cavity, but in one paper with 46 cases, 2 patients were classified as having minor salivary gland lipomas (Fregnani, Pires, and Falzoni et al. 2003).

Salivary lipomas usually present as slow-growing painless masses, and their appearance on CT or MR is diagnostic (Figure 11.6). Surgical excision is the treatment of choice, and although easy in classic lipoma, it can be challenging in the infiltrating variety (Figure 11.7).

Recently a designation of sialolipoma has been proposed for lipomas containing glandular elements, for example, ductal or acinar tissue (Nagao, Sugano, and Ishida et al. 2001). In their series of 2,051 salivary tumors, 7 sialolipomas, 5 in the parotid, and 2 palatal, were reported. Excision as for classic lipoma is curative. Since the initial report other cases both in major and minor glands have been published (Lin, Lin, and Chen et al. 2004; Michaelidis, Stefanopoulos, and Sam-baziotis et al. 2006).

Figure 11.6c. Intraoperative view of parotidectomy with parotid tail lipoma.

Figure 11.6a. Lipoma in the tail of the parotid.

Figure 11.6b. CT is diagnostic of lipoma.

Figure 11.6d. Specimen with arrows showing lipoma.

Figure 11.6e. Histopathology confirms the presence of lipoma.
Figure 11.7a. MR axial image of infiltrating lipoma of right submandibular region extending between the cervical muscles.

MALIGNANT MESENCHYMAL TUMORS Sarcomas

Sarcomas of the salivary glands are very rare and case reports of virtually all histologic types have been reported. In Siefert and Oehne's 1986 review of 167 mesenchymal tumors of the salivary glands, only 17 were sarcomas (10%). In this series, 5 cases were malignant fibrous histiocytomas, 5 cases were malignant schwannomas, 4 cases were embryonal rhabdomyosarcoma, and single cases of myxoid liposarcoma, leiomyosarcoma, and malignant hemangioendothelioma were reviewed. In reviewing salivary masses in children, rhabdo-myosarcomas were the most common malignant mesenchymal tumor (7%) (Bentz et al. 2000), and in 137 children with rhabdomyosarcomas of the head and neck the parotid was the site for 6% of these tumors (Hicks and Flaitz 2002). Obviously treatment plans will be dictated by the individual sarcoma type, with initial chemotherapy for rhab-domyosarcoma in children followed by radiation therapy or surgery for residual disease. Rhabdo-myosarcoma of the salivary glands appears locally aggressive with a poor prognosis (BenJelloun,

Figure 11.7b. Coronal MR image shows the lipoma extending medial to the right medial pterygoid muscle into the lateral pharyngeal space.

Jouhadi, and Maazouzi et al. 2005). In malignant fibrous histiocytoma, clear surgical margins appear to be the most important prognostic factor (Sachse, August, and Alberty 2006). Angiosarcoma may affect the parotid as a primary or metastatic tumor, and in a series of 29 angiosarcomas of the oral and salivary gland region there were 4 primary parotid and 3 primary submandibular gland angiosarco-mas with a further 3 metastatic to the parotid (Fanburg-Smith, Furlong, and Childers 2003).

All of the metastatic patients died, but patients with primary salivary gland angiosarcoma appear to have a better prognosis than those with cutaneous or deep tissue angiosarcomas. Malignant neural sarcomas are treated with wide excision and facial nerve grafting or reanimation (McGuirt, Johnson, and McGuirt 2003). Other sarcomas of the salivary glands are rare; for example, Chadan et al. (2004) found only 11 reported cases of salivary gland liposarcoma in the literature.

Sarcomas can involve any of the major salivary glands although the parotid is most common, and due to its rarity, treatment is usually on an individual and empiric basis (Figure 11.8).

Figure 11.8a. Rapidly growing sublingual gland tumor diagnosed as synovial cell sarcoma on biopsy and immunohistochemistry.

Figure 11.8c. CT bone window shows calcifications throughout the mass.

Figure 11.8b. CT image reveals calcification in the mass Figure 11.8d. Bilateral selective neck dissections in con-leading to an initial clinical diagnosis of a high-grade malig- tinuity with lip split to access the mandible. nant carcinoma ex-pleomorphic adenoma.
Figure 11.8e. Midline mandibulotomy prior to excision of Figure 11.8h. Four weeks post-surgery. the floor of mouth and ventral tongue.

Figure 11.8f. Surgical specimen.

-the reconstruction will be a

Figure 11.8g. Post-resection-microvascular forearm flap.

-the reconstruction will be a

Figure 11.8i. Intraoral view showing the forearm flap reconstruction of the floor of the mouth.

Figure 11.9a. Elderly man with primary desmoid melanoma of the parotid gland. A 2 cm margin is marked; the light blue staining of the skin around the lesion is from dye injection for sentinel node biopsy (patient had lymphoscin-tigraphy immediately preoperatively).

Figure 11.9c. The neck dissection and parotidectomy with preservation of the facial nerve is complete. The submental flap is pedicled on its vascular supply prior to being rotated into the defect.

Figure 11.9b. Markings for the proposed surgery involving a total parotidectomy with left supraomohyoid neck dissection (unless sentinel nodes are found at levels IV or V). Reconstruction with a submental flap based on the submental vessels.

Figure 11.9d. Three months postoperatively.

EPITHELIAL NON-SALIVARY TUMORS

The major salivary glands may be infiltrated by squamous cell carcinoma from the overlying skin or be primarily involved by melanoma. Surgical resection with a margin of normal tissue preserving the facial nerve and utilizing neck dissection and adjuvant radiotherapy as indicated by the tumor stage is the appropriate treatment (Figure 11.9).

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