SURGICAL ONCOLOGY AND RECONSTRUCTION
A New Clinical Manifestation for Cheek Alveolar Soft-Part Sarcoma: A Case Report and Review of the Literature Hong-wei Wang, MD,* Wei Dai, MD,y Xing-jun Qin, MD,z and Chen-ping Zhang, MDx Alveolar soft part sarcoma (ASPS) is a rare, histologically distinct, soft tissue malignancy with nonspecific clinical features usually described as a nonulcerated, painless, expanding mass. It has a pseudoalveolar appearance with clustered polygonal cells lacking central cohesion. It accounts for approximately 0.5 to 1% of all soft tissue sarcomas. It has a strong predilection for adolescents and young adults 15 to 35 years old, with a female predominance. In general, ASPS grows slowly, with a predilection for the trunk and extremities and rarely in the head and neck region. A literature review found only 11 cases of cheek ASPS that have been reported since 1952. This report describes the case of an unusually rapidly growing mass in the cheek of a 36-year-old woman. The superficial location of the mass led to early detection and treatment. Ó 2014 American Association of Oral and Maxillofacial Surgeons J Oral Maxillofac Surg 72:817-822, 2014 Alveolar soft-part sarcoma (ASPS) is a rare, histologically distinct, soft tissue malignancy with nonspecific clinical features usually described as a nonulcerated, painless, expanding mass.1 The name of the tumor was coined in 1952 by Christopherson et al2 because of the pseudoalveolar appearance with clustered polygonal cells lacking central cohesion. It accounts for approximately 0.5 to 1% of all soft tissue sarcomas.3 It has a strong predilection for adolescents and young adults 15 to 35 years old, with a female predominance.4-6 In general, ASPS grows slowly, with a predilection for the trunk and extremities,7 and is rare in the head and neck region. A literature review found only 11 cases of cheek ASPS that have been reported since 1952.1,8-16 This report describes the case of an unusually rapidly growing mass in the cheek of a 36-year-old woman. The superficial location of the mass led to early detection and beneficial treatment.
Report of Case
*Postgraduate, Department of Oral and Maxillofacial–Head and Neck Oncology, Ninth People’s Hospital, College of Stomatology,
Address correspondence and reprint requests to Dr Qin: Department of Oral and Maxillofacial-Head and Neck Oncology, Ninth
Shanghai Jiao Tong University School of Medicine, Shanghai, China.
People’s Hospital, College of Stomatology, Shanghai Jiao Tong
yAssociate Professor, Department of Oromaxillofacial–Head and
University School of Medicine, Shanghai 200011, People’s Republic
In May 2012, the patient presented with a rapidly enlarging painless swelling, measuring 0.5 cm in diameter, in the right buccal mucosa. An initial course of cephalosporin had no effect and over 2 weeks the tumor grew about 9 times its original size. She had no previous illnesses or subjective symptoms apart from fatigue and severe pain on palpation. She was admitted to the hospital on June 19, 2012, and on physical examination, the patient had a large swelling that infiltrated the right cheek area, the right corner of the mouth, and the upper and lower lips. The overlying skin was normal with no evidence of blanching, pulsations, or ulceration, but it was painful to palpation (Fig 1). The mouth opening of the patient had decreased. Intraoral examination showed a large
Neck Surgery, School of Stomatology, China Medical University,
of China; e-mail: [email protected]
Shenyang, Liaoning, China.
Received September 4 2013
zAssociate Professor, Department of Oral and Maxillofacial–Head
Accepted October 24 2013
and Neck Oncology, Ninth People’s Hospital, College of
Ó 2014 American Association of Oral and Maxillofacial Surgeons
Stomatology, Shanghai Jiao Tong University School of Medicine, Shanghai, China.
xProfessor, Department of Oral and Maxillofacial–Head and Neck Oncology, Ninth People’s Hospital, College of Stomatology, Shanghai Jiao Tong University School of Medicine, Shanghai, China.
CHEEK ALVEOLAR SOFT-PART SARCOMA
FIGURE 2. Wide excision of the entire tumor was performed with the patient under general anesthesia. The defection measured 9.0 7.0 cm, with tumor-free surgical margins. Wang et al. Cheek Alveolar Soft-Part Sarcoma. J Oral Maxillofac Surg 2014.
FIGURE 1. The patient had a large swelling that infiltrated the right cheek area, the right corner of the mouth, and the upper and lower lips. Wang et al. Cheek Alveolar Soft-Part Sarcoma. J Oral Maxillofac Surg 2014.
mass measuring 6.0 4.0 4.0 cm with multiple nodules, and the mass was firm on palpation and adherent to the cheek mucosa. The lesion extended into the buccinator and masseter muscles. There were no enlarged lymph nodes in the neck, supraclavicular, or axillary areas. On investigation, her hemogram and routine biochemistry results were normal. The clinical differential diagnosis was infection to the submasseteric space versus a benign mesenchymal neoplasm, such as hemangioma or lymphangioma. Ultrasonography disclosed a 6.0- 4.0- 2.7-cm well-defined hyperdense lesion in the right cheek that elevated the superior skin and mucosa. Computed tomographic scan showed a 6-cm soft tissue tumor in the right cheek extending into the buccinator and masseter muscles without a destructive process in the right mandibular ramus. Further ultrasonograms and thoracic radiographs showed no metastases in the lungs, liver, gallbladder, pancreas, spleen, and kidneys. According to the TNM system of the Union for International Cancer Control, this patient’s tumor was staged T3N0M0.
An excisional biopsy was performed under general anesthesia on June 20, 2012. It yielded a result of soft tissue sarcoma and suggested an immunohistochemical panel for definite diagnosis. Wide excision
FIGURE 3. An (anterolateral thigh) free flap measuring 15.0 7.0 cm was used for reconstruction. Wang et al. Cheek Alveolar Soft-Part Sarcoma. J Oral Maxillofac Surg 2014.
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FIGURE 4. This photograph was taken 14 months after surgery. The patient was clinically well with an improvement in appearance. Wang et al. Cheek Alveolar Soft-Part Sarcoma. J Oral Maxillofac Surg 2014.
of the entire tumor and a selective cervical lymph node dissection were performed on June 27, 2012. Radical resection was performed with 2-cm surgical margins
(Fig 2). The cutaneous defect and the mucosa and lips were repaired by an ALT (anterolateral thigh) free flap. As a result, the left and right sides were essentially symmetrical (Fig 3). Postoperatively, the patient underwent adjuvant radiotherapy for 25 days (total, 50 Gy). At the 14month follow-up, she was clinically well and had an improved appearance (Fig 4). There was no evidence of local recurrence or distant metastasis. The patient was still under regular follow-up. The surgical specimen was firm and of elastic consistency. Its cut surface was whitish and lobular. There was direct invasive growth into the muscles. Microscopically, the pseudoalveolar architecture was separated by thin well-vascularized fibrous septa (Fig 5). The tumor cells exhibited pleomorphism. The large polygonal cells contained abundant eosinophilic or translucent cytoplasm with 1 or 2 vesicular and eccentric nuclei. The nuclei were round to oval, with a distinct border and prominent nucleoli. Mitotic activity was scant (Fig 6). Immunohistochemically, the tumor cells were positive for periodic acid-Schiff (PAS; Fig 7), vimentin, myoglobin, CD34, and CD31, but negative for desmin, S-100, cytokeratin, Human Melanoma Black 45, and smooth muscle actin, which act as diagnostic markers for ASPS (Fig 8). These results supported the diagnosis of ASPS.
Discussion ASPS is a rare malignant soft tissue neoplasm with a strong predilection for adolescents and young adults
FIGURE 5. Low-power microscopy showed that the tumor was composed of a pseudoalveolar architecture that was separated by thin wellvascularized fibrous septa (hematoxylin and eosin stain; magnification, 40). Wang et al. Cheek Alveolar Soft-Part Sarcoma. J Oral Maxillofac Surg 2014.
CHEEK ALVEOLAR SOFT-PART SARCOMA
FIGURE 6. High-power microscopy showed that the tumor cells exhibited pleomorphism. Some large cells were round or polygonal, showing abundant eosinophilic or translucent cytoplasm with 1 or 2 vesicular and eccentric nuclei. The nuclei were round to oval, with a distinct border and prominent nucleoli. Some cells were small, showing evident nucleoli. Mitotic activity was scant (hematoxylin and eosin stain; magnification, 400). Wang et al. Cheek Alveolar Soft-Part Sarcoma. J Oral Maxillofac Surg 2014.
15 to 35 years old, with a female-to-male ratio of 1.5 to 2:1.3-6 It was first described in 1952 by Christopherson et al.2 Microscopically, ASPS is characterized by large polyhedral cells separated by delicate vascular channels and bands of fine connective tissue that confer an organoid pseudoalveolar architecture to the tumor.17 PASpositive diastase-resistant crystals in the cytoplasm have been found in more than 80% of cases. The PAS-positive results and the immunohistochemical
findings are diagnostic for ASPS.1,3 Although ASPS has a nonreciprocal chromosomal translocation, der(17) t(X;17)(p11;q25), with the corresponding oncogenic fusion gene, ASPL-TFE3, the origin of ASPS remains obscure.18 Despite very indolent growth, ASPS has a high propensity for metastasis. It most commonly spreads to the lungs in 42 to 65% of cases, and less common sites are the bones and the brain.7 ASPS can occur in any area of the body. The lower extremities are the most common primary sites.7 When it occurs in the head and neck, the most frequent locations are the tongue and orbit.16 Cheek ASPS is an extremely rare variation and only 11 cases have been reported thus far, which includes the present patient.1,8-16 The rarity of ASPS makes it difficult to draw definitive conclusions regarding its clinical characteristics. To the authors’ knowledge, this is the first case report of ASPS involving the cheek in which clinical growth was documented. In general, ASPS presents as a slow-growing mass.4-6 On analyzing the features of all previously reported cases of cheek ASPS, the authors found that the clinical history ranged from 1 to 24 months and averaged about 3 to 9 months. This is shorter than any ASPS cases in other parts of the body. The present case is unique because of the short clinical history. This patient’s neoplasm grew rapidly from 0.5 cm to 6.0 4.0 4.0 cm in less than 3 weeks. Such quick growth has never been reported. The variable growth rates and indolent nature of ASPS makes early detection difficult.6,7,19 However,
FIGURE 7. High-power microscopy showed positive results for Periodic acid-Schiff stain (arrows) (magnification, 400). Wang et al. Cheek Alveolar Soft-Part Sarcoma. J Oral Maxillofac Surg 2014.
WANG ET AL
FIGURE 8. Immunohistochemically, the tumor cells were positive for myoglobin (arrows) (magnification, 400). Wang et al. Cheek Alveolar Soft-Part Sarcoma. J Oral Maxillofac Surg 2014.
in the cheek, it is often diagnosed at an earlier stage because of the superficial location, easy visibility, and obvious functional impairment. The present patient was treated with radical excision and adjuvant radiotherapy, which resulted in a good clinical result at the 14-month follow-up. There was no evidence of local recurrence or distant metastasis, probably because of early radical excision and radiotherapy. The most commonly advocated and effective treatment for ASPS continues to be radical surgical excision of the tumoral tissue with 1.5- to 2-cm microscopically clear surgical margins.1,20 In patients with cheek ASPS, free flaps are used not only for reconstruction, but also for the improvement of quality of life. Routine adjuvant radiotherapy is essential for each patient.3,19 In the near future, antiangiogenic strategies may change the natural course of the disease and the possible approach to residual lesion and metastasizing disease.21,22 It also might be useful for presurgical debulking. In conclusion, the early clinical manifestations and treatment of cheek ASPS contributed to early detection and an ideal outcome. Despite more than 60 years of experience with ASPS, several fundamental questions regarding this tumor remain unanswered. Owing to the limited number of cases, far more investigations are needed to achieve greater knowledge.
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