Orbital alveolar soft part sarcoma: A report of 8 cases and review of the literature

Hei,Yan; Kang,Li; Yang,Xinji; Wang,Yi; Lu,Xiaozhong; Li,Yueyue; Zhang,Wei; Xiao,Lihua

doi:10.3892/ol.2017.7286

January-2018 Volume 15 Issue 1

Full Size Image

Journals

International Journal of Molecular Medicine

International Journal of Molecular Medicine is an international journal devoted to molecular mechanisms of human disease.

International Journal of Oncology

International Journal of Oncology is an international journal devoted to oncology research and cancer treatment.

Molecular Medicine Reports

Covers molecular medicine topics such as pharmacology, pathology, genetics, neuroscience, infectious diseases, molecular cardiology, and molecular surgery.

Oncology Reports

Oncology Reports is an international journal devoted to fundamental and applied research in Oncology.

Experimental and Therapeutic Medicine

Experimental and Therapeutic Medicine is an international journal devoted to laboratory and clinical medicine.

Oncology Letters

Oncology Letters is an international journal devoted to Experimental and Clinical Oncology.

Biomedical Reports

Explores a wide range of biological and medical fields, including pharmacology, genetics, microbiology, neuroscience, and molecular cardiology.

Molecular and Clinical Oncology

International journal addressing all aspects of oncology research, from tumorigenesis and oncogenes to chemotherapy and metastasis.

World Academy of Sciences Journal

Multidisciplinary open-access journal spanning biochemistry, genetics, neuroscience, environmental health, and synthetic biology.

International Journal of Functional Nutrition

Open-access journal combining biochemistry, pharmacology, immunology, and genetics to advance health through functional nutrition.

International Journal of Epigenetics

Publishes open-access research on using epigenetics to advance understanding and treatment of human disease.

Medicine International

An International Open Access Journal Devoted to General Medicine.

January-2018 Volume 15 Issue 1

Full Size Image

Article Open Access

Orbital alveolar soft part sarcoma: A report of 8 cases and review of the literature

Authors:
- Yan Hei
- Li Kang
- Xinji Yang
- Yi Wang
- Xiaozhong Lu
- Yueyue Li
- Wei Zhang
- Lihua Xiao
View Affiliations / Copyright

Affiliations: Institute of Orbital Disease, General Hospital of Chinese People's Armed Police Forces, Beijing 100039, P.R. China

Copyright: © Hei et al. This is an open access article distributed under the terms of Creative Commons Attribution License.
Pages: 304-314
|
Published online on: October 30, 2017

https://doi.org/10.3892/ol.2017.7286
Expand metrics +

Abstract

Alveolar soft part sarcoma (ASPS) is a rare soft tissue neoplasm. The incidence of orbital involvement is the highest in ASPS arising in the head and neck region. However, limited information is available regarding its clinical characteristics. The present study presents the clinical manifestations, imaging findings, pathological features, treatment strategies and prognosis records of 8 cases of orbital ASPS over the last 15 years in a single hospital, along with a review of the literature. Included were 3 male and 5 female patients, with the median age at presentation being 9.5 years. The mean average largest tumour diameter was 3.6 cm. A total of 5 patients underwent surgical excision of the tumour, with 2 undergoing orbital exenteration and 1 undergoing partial orbital exenteration. In total, 6 patients received postoperative radiotherapy and 2 received chemotherapy. Upon follow‑up, 6 patients were doing well with no evidence of recurrence or metastasis. Local recurrence developed in 2 patients, of whom 1 succumbed following withdrawal from treatment. According to the present series and the cases mentioned in the literature, orbital alveolar soft part sarcoma has characteristics distinct from those of alveolar soft part sarcoma which arises in other locations. Orbital alveolar soft part sarcoma presents itself in a younger population with a shorter course of disease, smaller tumour size, improved prognosis, a marked association with the extraocular muscles and with the Ki‑67 proliferation index possibly associated with prognosis of the disease.

View Figures

View References

1	Rose AM, Kabiru J and Rose GE: Alveolar soft-part sarcoma of the orbit. Afr J Paediatr Surg. 8:82–84. 2011. View Article : Google Scholar : PubMed/NCBI
2	Mulay K, Ali MJ, Honavar SG and Reddy VA: Orbital alveolar soft-part sarcoma: Clinico-pathological profiles, management and outcomes. J Cancer Res Ther. 10:294–298. 2014. View Article : Google Scholar : PubMed/NCBI
3	Kim HJ, Wojno T, Grossniklaus HE and Shehata BM: Alveolar soft-part sarcoma of the orbit: Report of 2 cases with review of the literature. Ophthal Plast Reconstr Surg. 29:e138–e142. 2013. View Article : Google Scholar : PubMed/NCBI
4	Majumdar K, Saran R, Tyagi I, Jain A, Jagetia A, Sinha S and Singh A: Cytodiagnosis of alveolar soft part sarcoma: Report of two cases with special emphasis on the first orbital lesion diagnosed by aspiration cytology. J Cytol. 30:58–61. 2013. View Article : Google Scholar : PubMed/NCBI
5	Rekhi B, Ingle A, Agarwal M, Puri A, Laskar S and Jambhekar NA: Alveolar soft part sarcoma ‘revisited’: Clinicopathological review of 47 cases from a tertiary cancer referral centre, including immunohistochemical expression of TFE3 in 22 cases and 21 other tumors. Pathology. 44:11–17. 2012. View Article : Google Scholar : PubMed/NCBI
6	Alkatan H, Al-Shedoukhy AA, Chaudhry IA and Al-Ayoubi A: Orbital alveolar soft part sarcoma: Histopathologic report of two cases. Saudi J Ophthalmol. 24:57–61. 2010. View Article : Google Scholar : PubMed/NCBI
7	Pang LJ, Chang B, Zou H, Qi Y, Jiang JF, Li HA, Hu WH, Chen YZ, Liu CX, Zhang WJ and Li F: Alveolar soft part sarcoma: A biomarker diagnostic strategy using TFE3 immunoassay and ASPL-TFE3 fusion transcripts in paraffin-embedded tumor tissues. Diagn Mol Pathol. 17:245–252. 2008. View Article : Google Scholar : PubMed/NCBI
8	Morris WR, Padgett DM, Osborn FD and Fleming JC: Pathologic quiz case: An orbital mass in a 45-year-old woman. Alveolar soft part sarcoma. Arch Pathol Lab Med. 129:534–536. 2005.PubMed/NCBI
9	Kanhere HA, Pai PS, Neeli SI, Kantharia R, Saoji RR and D'cruz AK: Alveolar soft part sarcoma of the head and neck. Int J Oral Maxillofac Surg. 34:268–272. 2005. View Article : Google Scholar : PubMed/NCBI
10	Kashyap S, Sen S, Sharma MC, Betharia SM and Bajaj MS: Alveolar soft-part sarcoma of the orbit: Report of three cases. Can J Ophthalmol. 39:552–556. 2004. View Article : Google Scholar : PubMed/NCBI
11	Khan AO and Burke MJ: Alveolar soft-part sarcoma of the orbit. J Pediatr Ophthalmol Strabismus. 41:245–246. 2004.PubMed/NCBI
12	Chan WM, Liu DT, Lai CK, Tse MW, Ng JS and Lam DS: Soft tissue sarcomas. Case 2. Orbital alveolar soft part sarcoma in a child. J Clin Oncol. 22:2027–2029. 2004. View Article : Google Scholar : PubMed/NCBI
13	Lasudry J and Heimann P: Cytogenetic analysis of rare orbital tumors: Further evidence for diagnostic implication. Orbit. 19:87–95. 2000. View Article : Google Scholar : PubMed/NCBI
14	Coupland SE, Heimann H, Hoffmeister B, Lee WR, Foerster MH and Gross U: Immunohistochemical examination of an orbital alveolar soft part sarcoma. Graefes Arch Clin Exp Ophthalmol. 237:266–272. 1999. View Article : Google Scholar : PubMed/NCBI
15	Chodankar CM, Pandit SP, Joshi MG and Deodhar KP: Alveolar soft-part sarcoma of the orbit (a case report). Indian J Ophthalmol. 34:67–68. 1986.PubMed/NCBI
16	Font RL, Jurco S III and Zimmerman LE: Alveolar soft-part sarcoma of the orbit: A clinicopathologic analysis of seventeen cases and a review of the literature. Hum Pathol. 13:569–579. 1982. View Article : Google Scholar : PubMed/NCBI
17	Ishikura A, Kimura A and Yamamoto S: A case of alveolar soft part sarcoma. Jpn J Cancer Clin. 25:6931979.
18	Mukherjee PK and Agrawal S: Alveolar soft part sarcoma of the orbit. Indian J Ophthalmol. 27:15–17. 1979.PubMed/NCBI
19	Varghese S, Nair B and Joseph TA: Orbital malignant non-chromaffin paraganglioma. Alveolar soft tissue sarcoma. Br J Ophthalmol. 52:713–715. 1968. View Article : Google Scholar : PubMed/NCBI
20	Abrahams IW, Fenton RH and Vidone R: Alveolar soft-part sarcoma of the orbit. Arch Ophthalmol. 79:185–188. 1968. View Article : Google Scholar : PubMed/NCBI
21	Altamirano-Dimas M and Albores-Saavedra J: Alveolar soft part sarcoma of the orbit. Arch Ophthalmol. 75:496–499. 1966. View Article : Google Scholar : PubMed/NCBI
22	Nirankari MS, Greer CH and Chaddah MR: Malignant non-chromaffin paraganglioma in the orbit. Br J Ophthalmol. 47:357–363. 1963. View Article : Google Scholar : PubMed/NCBI
23	Cheng Y, Sha Y, Huang W, Hong R and Cai R: CT and MRI features of orbital alveolar soft part sarcoma. Chin J Radiol. 47:1147–1148. 2013.(In Chinese).
24	Du C and Wu F: A case report of orbital alveolar soft part sarcoma and review of literatures. J ClinRadiol. 31:1814–1815. 2012.(In Chinese).
25	Feng N, Ma Z and Li C: Ultrasound features of a case of right orbital alveolar soft part sarcoma. Chin J Ultrasonogr. 19:6872010.(In Chinese).
26	Hu P, Zhou Z, Wang J, Peng W, Cheng Y, Zhu L and Tang F: Radiological features and clinicopathological findings of alveolar soft part sarcoma. Chin Oncol. 19:12–16. 2009.(In Chinese).
27	He W, Luo Q, Xia R and Yang G: Two cases of orbital relapsing alveolar soft part sarcoma. Chin J Ophthalmol. 40:848–850. 2004.(In Chinese).
28	Dong G, Wang J and Xie D: A case of intraorbital alveolar soft part sarcoma. Chin J Pract Ophthalmol. 22:3202004.(In Chinese).
29	Wang R and Wang W: A case of alveolar soft part sarcoma in orbit, sphenoidal, frontal, ethmoid and maxillary sinus. Chin Arch Otolaryngol Head Neck Surg. 10:2792003.(In Chinese).
30	Dong X, Wang L and Yan Q: Intraorbital alveolar soft part sarcoma. Chin J Diagnpathol. 3:1841996.(In Chinese).
31	Xu D: A case report of orbital alveolar soft part sarcoma. Guangdong Med J. 16:428–429. 1995.(In Chinese).
32	Li M, Zhang D and Yan H: Primary orbital alveolar soft part sarcoma. J Clin Ophthalmol. 1:123–124. 1993.(In Chinese).
33	Liu F: A case of orbital alveolar soft part sarcoma. Chin J Cancer. 4:3311989.(In Chinese).
34	Wei Y: A case of orbital alveolar soft part sarcoma. Chin J Ophthalmol. 21:2521985.(In Chinese).
35	Huo J, Xiong Q, Ni K and Wang R: Investigation of a case of orbital tumor. J Xinjiang Med University. 1:3221982.(In Chinese).
36	Ni C: Observation of ultrastructure of primary orbital alveolar soft part sarcoma. Chin J Ophthalmol. 17:5–7. 1981.(In Chinese).
37	Lieberman PH, Brennan MF, Kimmel M, Erlandson RA, Garin-Chesa P and Flehinger BY: Alveolar soft-part sarcoma. A clinico-pathologic study of half a century. Cancer. 63:1–13. 1989. View Article : Google Scholar : PubMed/NCBI
38	Casanova M, Ferrari A, Bisogno G, Cecchetto G, Basso E, De Bernardi B, Indolfi P, Bellani F Fossati and Carli M: Alveolar soft part sarcoma in children and adolescents: A report from the soft-tissue sarcoma italian cooperative group. Ann Oncol. 11:1445–1449. 2000. View Article : Google Scholar : PubMed/NCBI
39	Evans HL: Alveolar soft-part sarcoma. A study of 13 typical examples and one with a histologically atypical component. Cancer. 55:912–917. 1985. View Article : Google Scholar : PubMed/NCBI
40	Fanburg-Smith JC, Miettinen M, Folpe AL, Weiss SW and Childers EL: Lingual alveolar soft part sarcoma; 14 cases: Novel clinical and morphological observations. Histopathology. 45:526–537. 2004. View Article : Google Scholar : PubMed/NCBI
41	Ordóñez NG: Alveolar soft part sarcoma: A review and update. Adv Anat Pathol. 6:125–139. 1999. View Article : Google Scholar : PubMed/NCBI
42	Pennacchioli E, Fiore M, Collini P, Radaelli S, Dileo P, Stacchiotti S, Casali PG and Gronchi A: Alveolar soft part sarcoma: Clinical presentation, treatment, and outcome in a series of 33 patients at a single institution. Ann Surg Oncol. 17:3229–3233. 2010. View Article : Google Scholar : PubMed/NCBI
43	Reis H, Hager T, Wohlschlaeger J, Bauer S, Katenkamp K, Katenkamp D and Baba HA: Mammalian target of rapamycin pathway activity in alveolar soft part sarcoma. Hum Pathol. 44:2266–2274. 2013. View Article : Google Scholar : PubMed/NCBI
44	Khanna P, Paidas CN and Gilbert-Barness E: Alveolar soft part sarcoma: Clinical, histopathological, molecular, and ultrastructural aspects. Fetal Pediatr Pathol. 27:31–40. 2008. View Article : Google Scholar : PubMed/NCBI
45	Viry F, Orbach D, Klijanienko J, Fréneaux P, Pierron G, Michon J, Neuenschwander S and Brisse HJ: Alveolar soft part sarcoma-radiologic patterns in children and adolescents. Pediatr Radiol. 43:1174–1181. 2013. View Article : Google Scholar : PubMed/NCBI
46	Chen YD, Hsieh MS, Yao MS, Lin YH and Chan WP: MRI of alveolar soft part sarcoma. Comput Med Imaging Graph. 30:479–482. 2006. View Article : Google Scholar : PubMed/NCBI
47	Chu WC, Howard RG, Roebuck DJ, Chik KW and Li CK: Periorbital alveolar soft part sarcoma with radiologic features mimicking haemangioma. Med Pediatr Oncol. 41:145–146. 2003. View Article : Google Scholar : PubMed/NCBI
48	Folpe AL and Deyrup AT: Alveolar soft-part sarcoma: A review and update. J Clin Pathol. 59:1127–1132. 2006. View Article : Google Scholar : PubMed/NCBI
49	Enzinger FM and Weiss SW: Soft tissue tumors. 3rd. St Louis: C. V. Mosby; pp. 1067–1074. 1995
50	Portera CA Jr, Ho V, Patel SR, Hunt KK, Feig BW, Respondek PM, Yasko AW, Benjamin RS, Pollock RE and Pisters PW: Alveolar soft part sarcoma: Clinical course and patterns of metastasis in 70 patients treated at a single institution. Cancer. 91:585–591. 2001. View Article : Google Scholar : PubMed/NCBI
51	Miettinen M and Ekfors T: Alveolar soft part sarcoma. Immunohistochemical evidence for muscle cell differentiation. Am J Clin Pathol. 93:32–38. 1990. View Article : Google Scholar : PubMed/NCBI
52	Rosai J, Dias P, Parham DM, Shapiro DN and Houghton P: MyoD1 protein expression in alveolar soft part sarcoma as confirmatory evidence of its skeletal muscle nature. Am J Surg Pathol. 15:974–981. 1991. View Article : Google Scholar : PubMed/NCBI
53	Foschini MP and Eusebi V: Alveolar soft-part sarcoma: A new type of rhabdomyosarcoma? Semin Diagn Pathol. 11:58–68. 1994.PubMed/NCBI
54	Wang NP, Bacchi CE, Jiang JJ, McNutt MA and Gown AM: Does alveolar soft-part sarcoma exhibit skeletal muscle differentiation? An immunocytochemical and biochemical study of myogenic regulatory protein expression. Mod Pathol. 9:496–506. 1996.PubMed/NCBI
55	Gómez JA, Amin MB, Ro JY, Linden MD, Lee MW and Zarbo RJ: Immunohistochemical profile of myogenin and MyoD1 does not support skeletal muscle lineage in alveolar soft part sarcoma. Arch Pathol Lab Med. 123:503–507. 1999.PubMed/NCBI
56	Stockwin LH, Vistica DT, Kenney S, Schrump DS, Butcher DO, Raffeld M and Shoemaker RH: Gene expression profiling of alveolar soft-part sarcoma (ASPS). BMC Cancer. 9:222009. View Article : Google Scholar : PubMed/NCBI
57	Kayton ML, Meyers P, Wexler LH, Gerald WL and LaQuaglia MP: Clinical presentation, treatment, and outcome of alveolar soft part sarcoma in children, adolescents, and young adults. J Pediatr Surg. 41:187–193. 2006. View Article : Google Scholar : PubMed/NCBI
58	Sherman N, Vavilala M, Pollock R, Romsdahl M and Jaffe N: Radiation therapy for alveolar soft-part sarcoma. Med Pediatr Oncol. 22:380–383. 1994. View Article : Google Scholar : PubMed/NCBI
59	Jia Y, Wu D, Shang C, Yu J and Zhang KR: Alveolar soft part sarcoma occurring on the abdominal wall of a 2-year-old child. J Pediatr Hematol Oncol. 33:e80–e82. 2011. View Article : Google Scholar : PubMed/NCBI
60	Pappo AS, Parham DM, Cain A, Luo X, Bowman LC, Furman WL, Rao BN and Pratt CB: Alveolar soft part sarcoma in children and adolescents: Clinical features and outcome of 11 patients. Med Pediatr Oncol. 26:81–84. 1996. View Article : Google Scholar : PubMed/NCBI