Spindle cell squamous carcinoma (SpCC) is an exceedingly rare variant of squamous cell carcinoma with unique clinicopathological characteristics. SpCC is an aggressive and rapidly progressive neoplasm, and increased awareness for dentists, oral surgeons, and head and neck specialists should aid diagnosis and prompt treatment. We present a case of SpCC in the anterior maxilla of an 84-year-old female which displayed rapid growth.
SpCC is an exceedingly rare variant of squamous cell carcinoma.
SpCC is an aggressive and rapidly progressive neoplasm.
SpCC is biphasic, meaning it has both an epithelial and mesenchymal component.
Many important differences exist between SpCC and SCC.
SpCC is a more aggressive variant of SCC.
Spindle cell squamous carcinoma (SpCC) is an exceedingly rare variant of squamous cell carcinoma (SCC) with unique clinicopathological characteristics. The World Health Organization (WHO) recognizes SpCC as a variant of SCC, and includes the term sarcomatoid squamous cell carcinoma [ ]. Several other terminologies have also been used including polypoid squamous cell carcinoma, pseudo sarcoma, carcinosarcoma, collusion tumor, and pleomorphic carcinoma [ , ]. SpCC is often referred to as a biphasic malignant neoplasm, describing both an epithelial SCC component and a mesenchymal sarcomatoid spindle cell component [ ].
SpCC may arise from numerous areas throughout the body, such as the aerodigestive tract, salivary glands, breast, skin, urogenital tract, and gastrointestinal tract [ ]. In the head and neck region, the larynx is the predominant site of occurrence followed by the oral cavity, pharynx, and sinonasal tract. In the oral cavity, SpCC compromises less than 1% of all oral cavity tumors, and it has a site predilection for the alveolar mucosa, tongue, buccal mucosa, and lower lip [ ].
SpCC exhibits similar demographics to the conventional squamous cell carcinoma, with a higher prevalence in males and wider age range of occurrence, but primarily in the fifth and sixth decades of life [ ]. Tobacco use, alcohol consumption, trauma, and history of radiation therapy are presumed to be strongly associated with the development of SpCC.
In the report below, we present a case of SpCC of the anterior maxilla in an 84-year-old female which had a rapid growth.
An 84-year-old female presented to our clinic in December 2018 with pain and an expanding mass in her anterior maxilla. In April 2018, the patient had seen her dentist and was diagnosed with pemphigus vulgaris via incisional biopsy. The dentist also noted mobility of both central maxillary incisors, which worsened at the subsequent visit. In June, teeth #6–10 were extracted and a 7-unit temporary bridge was placed while the permanent bridge was sent for fabrication. The patient underwent cataract surgery shortly after, which postponed delivery of the bridge, as well as pemphigus vulgaris treatment. At the follow-up in November, significant erythema was noted beneath the temporary bridge, and a radiograph showed worsening vertical bone loss. Referral to a community oral surgeon was made, and the patient was immediately sent to our clinic with a high concern for malignancy.
Upon presentation, the patient reported a 5kg weight-loss. Medical history was significant for breast cancer treated with radiation and bilateral mastectomy over a decade prior. She smoked ½ pack of tobacco for a 5-year period in her 20’s.
On exam, a wide-based, erythroleukoplakic lesion in the anterior maxilla was expanding beneath the temporary fixed partial denture. The lesion was friable, poorly demarcated, extending from the vestibule to the soft palate. Biopsy showed SpCC versus low grade epithelial malignancy.
Imaging showed a 2.26 cm by 1.93 cm by 1.51 cm hyperdense mass causing erosion of the anterior maxillary alveolus, superiorly to the right piriform rim. Palatal bone had minimal erosion, sinuses were clear. No lymphadenopathy was present in the neck, and chest imaging was negative.
An anterior partial maxillectomy was performed in January 2019. Teeth #1, 14, and 15 remained, and stabilized a pre-fabricated stent using COE-SOFT ®, wires and screws. Ten frozen sections showed negative margins. The patient had an uneventful post-op course. The final pathology was a T4aN0M0 SpCC. At time of resection the lesion was 6.2 cm × 4.5 cm x 2.1cm.
After discharge, the patient and family reported oral ulcerations and blisters initially thought to be traumatic. The blisters continued growing and developed appearance similar to the primary tumor. By mid-February, approximately 4 growths displaced the obturator, which was re-aligned, but was eventually displaced again. At follow up, lymphadenopathy of the neck was present bilaterally and the service discussed bilateral neck dissection and radiation therapy with the patient and family. The decision was made to forego further treatment. The patient was accepted to hospice care and passed away one month later.
The tumor was a unifocal mass (6.2 cm × 4.5 cm x 2.1cm) at the maxillary midline. Sections of the mass showed multiple areas of spindle cell proliferation with a myxoid background of elongated, spindled fibroblasts in loosely arranged whorls. Cells showed mild to moderate cytologic atypia with abundant mitotic activity with rare atypical mitoses ( Figs. 1–4 ).