Epidermoid carcinoma involving the parotid gland and orbital region: case report of a rare entity.
Epidermoid carcinoma (also known as squamous cell carcinoma) of the parotid gland is more prevalent among the elderly, as are other head and neck tumors. In most cases it is observed either as a nodal metastasis of a primary tumor that drains into the parotid area or as a direct extension of a tumor that has originated in the upper dermis. Primary epidermoid carcinomas arising directly from the parotid gland are extremely rare. Similarly, secondary invasion of the orbital region by a neighboring tumor is more common than primary epidermoid carcinoma of the orbit, which is also rare. In this article, we discuss the diagnosis and treatment of a rare case of epidermoid carcinoma simultaneously invading the parotid gland and the orbit.
Although primary epidermoid carcinoma, also known as squamous cell carcinoma (SCC), of the parotid gland is quite rare, it presents an important clinical problem because of its rapid progression and aggressive course. The parotid gland is a common location to which cutaneous tumors of the head and neck metastasize. If an epidermoid carcinoma of the scalp, face, eyelid, conjunctiva, malar region, radix of the nose, upper lip, anterior ear, external ear canal, or preauricular or postauricular region occurs simultaneously with an epidermoid carcinoma of the parotid gland, metastasis from those regions should be suspected rather than a primary parotid gland lesion. (1-5) However, mucoepidermoid carcinomas and parotid metastases of epidermoid carcinomas are often confused with primary epidermoid carcinomas of the parotid gland.
In a similar fashion, epidermoid carcinoma of the orbital region is usually observed as a secondary invasion of a tumor from a neighboring structure. Most patients with this tumor are of advanced age. (6,7) Early diagnosis and proper treatment are necessary because this tumor can cause blindness and life-threatening complications. Although rarely, epidermoid carcinoma of the eyelid and conjunctiva also can metastasize to the parotid lymph nodes. (1,3)
An 89-year-old woman came to our clinic with a lump and pain in front of her right ear of 1 year's duration. Upon examination, a tender, immobile mass was detected in the right preauricular region. It was hard in consistency and measured 4 x 3 cm by palpation. The skin covering the mass was hyperemic, raising the suspicion of tumoral infiltration. A solid, fixed mass also was noted on the patient's right upper eyelid. Its margins were unidentifiable because of intense mucopurulent discharge and hyperemia. The patient's visual acuity could not be assessed because she could not open the affected eye. Her medical history included cataract surgery on the same eye 5 years earlier, with unsatisfactory vision postoperatively. Her eyes had been examined and, apart from the vision difficulty on the operated side, evaluated as normal 1 year before she presented at our clinic.
Computed tomography (CT) (figure 1, A) and magnetic resonance imaging (MRI) (figure 1, B) revealed a 3 x 3-cm soft-tissue lesion on the right parotid gland, with margins indistinguishable from the masseter muscle. The lesion showed peripheral contrast enhancement and contained central cystic and necrotic areas.
[FIGURE 1 OMITTED]
Orbital CT showed an intact bulbus oculi and normal intraocular structures, but on the superior aspect of the right eyelid, a soft-tissue thickening of 1 cm with sporadic nodularity and peripheral contrast enhancement was detected (figure 2). Homogeneous contrast enhancement on the right lacrimal gland also was noted. There was no evidence of distant metastasis.
An eye examination performed under sedation revealed that the upper eyelid was infiltrated with a soft, fragile, and lobulated mass. The tumor was observed to be encasing the globe from the superior, inferior, and temporal aspects, as well as infiltrating the sclera and conjunctiva. The superior portion of the sclera was observed to be moderately fragile. With manual examination, the mobility of the globe was found to be limited. A fine-needle aspiration (FNA) biopsy of the parotid gland and incisional biopsies (as the tumor infiltrated the skin) of the parotid gland and orbital region were taken. The FNAbiopsyofthe parotid gland demonstrated poorly differentiated mucoepidermoid carcinoma and/or undifferentiated carcinoma. Histochemical analysis of the obtained specimens indicated a malignant neoplastic growth with high mitotic activity. The growth was noted to be forming cords and islets within the desmoplastic stroma, with hyperchromic, large nuclei and prominent nucleoli. There was also sparse singlecell hyperkeratinization. No histochemical staining was seen with periodic acid-Schiff (PAS) or mucicarmine stains, which were performed to further elucidate the diagnosis. The morphologic and histochemical findings indicated a poorly differentiated epidermoid carcinoma (figure 3).
[FIGURE 2 OMITTED]
Exenteration of the right eye, a radical parotidectomy, and radical neck dissection were performed. Pathologic examination of the specimen revealed the apex of the exenteration and the surgical borders to be tumor-free; however, the examination showed that the tumor had invaded the facial nerve and vascular structures and that there was extensive tumor growth inside the orbit.
The patient underwent postoperative radiation therapy, which was directed at the surgical area. At 7 months postoperatively, a recurrent mass posterolateral to the sternocleidomastoid muscle was detected. The patient was hospitalized because of feeding difficulties and refused the offered chemotherapy. She died 2 months later as a result of multiple metastases.
Parotid tumors. Malignant parotid tumors represent 3 to 12% of head and neck malignancies? Several researchers have noted that the incidence of primary epidermoid carcinoma of the parotid is lower than that of metastatic epidermoid carcinoma. (2,5,8-10) Primary epidermoid carcinomas of the parotid constitute 0.1 to 1.8% of all parotid gland tumors, (5,10) while metastatic epidermoid carcinomas constitute 1.2 to 4.6%. (3,911)
[FIGURE 3 OMITTED]
Lesions of the head and neck region have the potential to invade the parotid gland because of the complex lymphatic network of the parotid and its rich interconnections with surrounding regions. The involvement of parotid nodes, either by direct extension or by metastasis, is a serious problem. If the tumor extends out with the nodes into the gland, the prognosis is poor, even after radical surgery. This is especially true of metastatic tumors, whose extension is rapid and which have high mortality and morbidity rates. (4)
Most tumors metastasizing to the parotid nodes originate in the skin. Usually, ipsilateral tumors of the lateral and frontal scalp, face, eyelid, conjunctiva, malar region, radix of the nose, upper lip, anterior ear, external ear canal, or preauricular or postauricular region metastasize to the parotid. (1-4) Therefore, it is important to examine the skin, especially the haired skin, of a patient with a parotid mass.
Although primary epidermoid parotid tumors are rare, they can easily be confused with more common mucoepidermoid carcinomas or metastatic epidermoid carcinomas of the parotid. (9) Batsakis et al reported 14 primary epidermoid carcinoma cases in their series of 580 parotid tumors. (9) They later reviewed the clinical and histopathologic records and reclassified the tumors. At that time they stated that only 2 of the 14 cases had in fact been primary epidermoid carcinomas of the parotid; 5 had been mucoepidermoid carcinomas, and 7 had been metastatic carcinomas originating from the oropharynx, oral cavity, and the skin of the lateral part of the face. (9) In general, if an epidermoid carcinoma is found in the ipsilateral auricular region, on the scalp, on the eyelid, or on the skin of the postauricular region, the parotid lesion should be considered metastatic rather than primary. (3,12)
In reporting on their series of 30 cases of epidermoid carcinoma of the parotid, Marks et al stated that 3 of the cases resulted from direct invasion of the skin covering the parotid and 24 originated at other skin sites that drain into the parotid area. (2) They identified only 3 cases of primary epidermoid carcinoma of the parotid.
A combination of surgery and radiotherapy is advocated in metastatic involvement of the parotid gland. (3,13) Because there are no established criteria to allow the surgeon to predict whether a cutaneous tumor will metastasize to the parotid, (4) the main issue in dealing with those kinds of tumors should be based on the tumor's potential for rapid, aggressive spread.
The type of parotidectomy performed should be based on the disease's clinical involvement, as follows:
* If the involved lymph node is mobile and located superficially and the tumor is confined to the node's borders, a superficial parotidectomy, sparing the facial nerve, is usually performed.
* If neural and diffuse glandular involvement is present, then radical excision with facial nerve sacrifice is performed.
* If clinical and/or metastatic disease extends deep into the facial nerve, radical total parotidectomy is performed.
* When the skin is also invaded by the tumor, the skin should be included in the surgical specimen and the resulting defect reconstructed with a flap.
* If any palpable lymph node exists in the neck, modified radical neck dissection should be carried out.
* If the neck is clinically negative, selective neck dissection or radiotherapy is the best choice.
A study by Lee et al demonstrated that patients with metastases to the parotid gland who presented a short time (mean: 4 months) after excision of a primary tumor tended to have lymph node involvement that could be treated with superficial parotidectomy. (4) Patients with a longer delay (mean: 8 months) tended to have more diffuse involvement of the parotid gland and required total parotidectomy. (4)
Marks et al reported that, in their series, all patients with metastases to the parotid gland presented within 24 months after excision of their skin lesions. Like Lee et al, these researchers found that those patients who presented with metastases to the parotid 8 months or more after treatment for the primary skin cancer tended to have more advanced disease. Lee et al also noted that the risk of metastasis from skin cancer to the parotid decreases over time but remains significant until about 19 months. (4) Marks et al suggested that patients be followed monthly for the first 2 years after skin cancer treatment and closely thereafter. (2) It also has been stated that an overall incidence of 1.5% of metastases of skin cancer to the parotid nodes is too low to advocate superficial parotidectomy in all patients. (2) Even if that is true, these patients need to be followed very closely after excision of their skin cancers.
Orbital tumors. It should be noted, in light of our patient's having epidermoid carcinoma involving both the parotid gland and the orbital region, that although many epidermoid tumors originating in the pinna and metastasizing to the parotid gland have been reported, the incidence of orbital tumors metastasizing to the parotid gland cannot be determined because this is such a rare occurrence. However, as we noted earlier, epidermoid carcinomas of the eyelid do have a tendency to metastasize to the parotid gland. (1,4)
Orbital tumors can be primary, secondary, or metastatic. An indurated, palpable periocular mass, an ulcerated lesion, and ocular motility disturbances are the most frequent findings at initial examination. (6) Primary epidermoid carcinoma of the orbit results from SCC of the lacrimal gland, which is a rare entity. SCCs of the lung and salivary glands occasionally metastasize to the orbit. In fact, most orbital SCCs are secondary. Secondary orbital tumors result from direct tumor extension from adjacent structures, such as the eyelid, conjunctiva, globe, intracranial cavity, or paranasal sinuses. In Western countries, most secondary SCCs extend to the orbit from the paranasal sinuses or eyelid skin; in tropical countries, orbital involvement of conjunctival epidermoid tumors is more common. (14)
Donaldson et al reported 3 cases of orbital invasion in their series of 51 epidermoid carcinomas of the eyelid. (15) Similarly, Howard et al detected only 2 cases of orbital invasion out of 622 eyelid epidermoid carcinomas. (6) The mean time interval between the eyelid carcinoma diagnosis and the detection of orbital invasion was nearly 1 year in both studies.
McKelvie et al noted 4 cases of orbital invasion in their series of 26 cases of epidermoid carcinoma of the conjunctiva. (7) Iliff et al reported 4 of 27 (14.8%) cases of epidermoid carcinoma with orbital invasion, (16) and Tunc et al reported that 11% of patients with epidermoid carcinoma had orbital invasion. (17) Johnson et al reported that 28 of 125 (22.4%) orbital tumors had conjunctival epidermoid carcinoma involvement. (14) The high prevalence of SCC of the orbit in the tropical regions mentioned in the latter study may be related to several factors, including continuous exposure to ultraviolet rays, chronic irritation, inflammation, and other factors, such as papillomavirus infection. (14) Conjunctival SCC is an aggressive disease in tropical and subtropical regions, where it tends to occur in younger patients.
Large or recurrent tumors in the periocular region should be treated aggressively at the early stages of the disease, to prevent vision loss secondary to orbital invasion and intracranial spread. Once a diagnosis of epidermoid carcinoma of the conjunctiva or the eyelid has been made, wide surgical excision of the tumor, using frozen-section control to ensure tumor-flee margins, should be performed. If orbital extension has occurred, exenteration is usually required, as well as removal of any involved bone or sinuses, because of the infiltrative nature of the tumor. Lymph node dissection is used for regional lymph node metastases. In patients with advanced metastases, such as asymptomatic microscopic metastases or clinical perineural involvement, adjuvant radiotherapy may be required. (14)
In our case, both orbital and parotid involvement occurred. The patient presented with complaints of swelling and extreme pain in her right preauricular area and right eye, as well as mucopurulent discharge from the eye. She was unable to open her eye and was experiencing vision difficulties. The tumor was suspected to be caused by chronic inflammation secondary to the patient's earlier cataract surgery. She had experienced vision problems ever since that surgery, even though an eye examination she underwent 1 year before presenting to us was normal. As the definitive histopathologic discrimination could not be made as to whether the tumor in her parotid gland was primary or metastatic, we speculated that the coexistence of anipsilateral epidermoid carcinoma of the parotid gland should raise the possibility of an epidermoid tumor stemming from the eyelid or conjunctiva, which drains into this region's lymph nodes.
Despite postoperative radiotherapy, our patient's disease progressed rapidly, and recurrence was detected 7 months postoperatively. She refused further therapy and died 2 months later.
The occurrence of an epidermoid carcinoma of the parotid gland should alert the physician to the possibility that the tumor stems from an epidermoid carcinoma of a neighboring structure, such as the eyelid or conjunctiva. Epidermoid carcinoma of the parotid gland has a more aggressive course than other epidermoid carcinomas of the head and neck. The prognosis depends on whether the tumor is confined to the intraglandular node or has spread to the parenchyma. The prognosis is relatively good when the former occurs but is extremely poor when the latter is the case, even despite radical surgery. (4) Therefore, it is crucial to examine other possible primary focuses that might be the source of the tumor when a patient presents with a parotid mass.
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(14.) Johnson TE, Tabbara KF, Weatherhead RG, et al. Secondary squamous cell carcinoma of the orbit. Arch Ophthalmol 1997;115(1):75-8.
(15.) Donaldson MJ, Sullivan TJ, Whitehead KJ, Williamson RM. Squamous cell carcinoma of the eyelids. Br J Ophthalmol 2002;86 (10):1161-5.
(16.) Iliff WJ, Marback R, Green WR. Invasive squamous cell carcinoma of the conjunctiva. Arch Ophthalmol 1975;93(2): 119-22.
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Sundus Aslan, MD; Haluk Yavuz, MD; Rana Altan-Yaycioglu, MD; Filiz Bolat, MD; Nuri Ozgirgin, MD
From the Department of Otorhinolaryngology (Dr. Asian, Dr. Yavuz, and Dr. Ozgirgin), the Department of Ophthalmology (Dr. Altan-Yaycioglu), and the Department of Pathology (Dr. Bolat), Baskent University School of Medicine, Ankara, Turkey.
Corresponding author: Sundus Asian, MD, Baskent Universitesi, Adana Seyhan Hastanesi, Baraj Yolu 1 Durak, KBB Klinigi Seyhan, Adana, Turkey. Phone: 90-533-634-6333; fax: 90-322-459-9197; e-mail: firstname.lastname@example.org
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|Title Annotation:||ORIGINAL ARTICLE|
|Author:||Aslan, Sundus; Yavuz, Haluk; Altan-Yaycioglu, Rana; Bolat, Filiz; Ozgirgin, Nuri|
|Publication:||Ear, Nose and Throat Journal|
|Article Type:||Case study|
|Date:||Jul 1, 2008|
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