Gaitan-Gaona et al., Clin Dermatol Res J 2016, 1:1
Clinical Dermatology Research Journal Case Report
a SciTechnol journal
Primary Synovial Sarcoma of the Scalp: An Unusual Presentation
was fixed in 10% buffered formalin and embedded in paraffin. Histologic 4 micras sections were stained by hematoxylin and eosin. Immunohistochemistry was performed using the standard streptavidin–biotin complex method.
Abstract
Immunohistochemical examination revealed that the cells were positive for TLE-1, Bcl2, Vimentin and CD-99 (Figure 3A-D). The proliferative activity as demonstrated by Ki67 staining was up to 50% on neoplastic cells. All neoplastic cells were negative for SMA, Desmin, EMA, S-100 and CD-34.
Francisco Gaitan-Gaona *, Mirra C Said , Mario Aurelio Martinez-Jimenez2 and Rodrigo Valdes-Rodriguez3 1
3
Synovial sarcomas are a rare subset of malignant soft tissue tumors, with an estimated incidence of 2.75 per 100,000 in the general population. Approximately 60% of all synovial sarcomas arise in the lower limbs, more specifically the thigh. On occasion (3-5%), the tumor is found to arise in the head and neck; synovial sarcoma on the scalp is a rare finding. We present a case of primary synovial sarcomaconfirmed by both histology and immunohistochemistry, arising from the scalp in an 18 year old male. Keywords Synovial sarcoma; Scalp; Immunohistochemical
Introduction Synovial sarcomas represent a subset of malignant soft tissue tumors. The name synovial sarcoma, a relic of 20th century medicine, is a misnomer. Indeed, the tumor is unrelated to the synovium and can occur anywhere in the body. With an estimated incidence of 2.75 per 100,000 in the general population, synovial sarcomas are rare; accounting for approximately 6-10% of all sarcomas, which as a class, account for less than 1% of all malignant tumors in adults [1,2]. Approximately 60% of all synovial sarcomas arise in the lower limbs, especially in the thigh. On occasion (3-5%), the tumor is found to arise in the head and neck, but on the scalp is a rare finding with only one case reported in the English language literature [1-4]. We describe the histopathological findings and immunohistochemical expression of a primary synovial sarcomaarising from the scalp of an 18 year old male.
Case Report An 18 year old male presented with 3 years history of a growing tumor on the right parietal region. A physical examination showed an ulcerated scalp tumor. There were no cervical lymphadenomegalies. The tumor was surgically resected with free margins and rotation flap to cover the wound.
The histology showed monophasic synovial sarcoma with spindleshaped cells arranged in a storiform pattern (Figure 2A), the cells have uniform appearance with small amounts of indistinct cytoplasm and oval dark-staining nuclei (Figure 2B). The mitotic index was 8/10 high power fields.
Outcome Chemotherapeutic and radiotherapeutic treatment was initiated after surgical resection and subsequent tissue diagnosis. The patient received 20 Gy of radiotherapy, follow by a total of 11 cycles of chemotherapy with gemzar and paclitaxel, at which point the patient was found to be in remission.
Discussion The synovial sarcoma is malignant soft tissue tumor arising predominantly in the lower extremities; only 3-5% of all cases occur in the head and neck region. Synovial sarcoma arising from the scalp
1A
1B
Figure 1: (A)-An ulcerated scalp tumor on the right parietal region. (B)-The cut surface of the tumor is white-tan and solid with hemorrhagic areas.
2A
2B
Grossly, the tumor measured 5 × 5 × 3.5 cm with an ulcerated surface and firm consistency (Figure 1). The cut surface of the tumor was white tan and solid with hemorrhagic areas. The specimen *Corresponding author: Francisco Gaitan-Gaona, Department of Pathology, Hospital General de Soledad Prolongación Valentine Amador 1112, Col. Rivas Guillen, San Luis Potosí, México- CP78432, Tel: 4445805578; E-mail:
[email protected] Received: June 14, 2016 Accepted: September 02, 2016 Published: September 08, 2016
International Publisher of Science, Technology and Medicine
Figure 2: (A)-Spindle-shaped cells in a stori form pattern (H/E). (B)-Cells with uniform appearance with small amounts of indistinct cytoplasm and oval darkstaining nuclei.
All articles published in Clinical Dermatology Research Journal are the property of SciTechnol, and is protected by copyright laws. Copyright © 2016, SciTechnol, All Rights Reserved.
Citation: Gaitan-Gaona F, Said MC, Martinez-Jimenez MA, Valdes-Rodriguez R (2016) Primary Synovial Sarcoma of the Scalp: An Unusual Presentation.Clin Dermatol Res J 1:1.
3A
3B
TLE-1 antibody a very important tool for the diagnosis of synovial sarcoma. In summary, synovial sarcoma on the scalp is a rare malignancy. In clinical practice the correlation of the histology with a panel of immunohistochemical markers make its diagnosis possible with certainty, even in unusual locations. References 1. Deshmukh R, Mankin HJ, Singer S (2004) Synovial sarcoma: the importance of size and location for survival. Clin Orthop Relat Res 419: 155-161.
3C
3D
2. Burningham Z, Hashibe M, Spector L, Joshua D (2012). The epidemiology of sarcoma. Clin Sarcoma es 2: 14. 3. Rangheard AS, Vanel D, Viala J, Schwaab G, Casiraghi O, et al. (2001) Synovial sarcoma of the head and neck: CT and MR imaging findings of eight patients. Am J Neuroradiol. 22: 851-857. 4. Al-Daraji W, Lasota J, Foss R, Miettinen M (2009) Synovial sarcoma involving the head: analysis of 36 cases with predilection to the parotid and temporal regions. Am J Surg Pathol 33: 1494-1503.
Figure 3: (A)-Neoplastic cells show a strong and diffuse nuclear reactivity for TLE-1 (Immunohistochemistry). (B)-Neoplastic Cells show diffuse cytoplasmic reactivity for Bcl-2 (Immunohistochemistry). (C)-A subset of tumor cells shows weak cytoplasmic and membrane staining for CD-99 (Immunohistochemistry). (D)-Strong, diffuse cytoplasmic reactivity for Vimentin is observed in tumor cells (Immunohistochemistry).
is an extremely rare presentation [1,4]. To the best of our knowledge, this case report represents the second documented case in the English medical literature. Wael Al-Daraji et al. first reported the only other instance in theiranalysis of 1,935 cases of synovial sarcomas from all sites; 36 of whichwere found to arise from the head and neck, with only one case arising from the scalp [4]. Microscopically, synovial sarcoma can be classified into four types: biphasic type, monophasic epithelial type, monophasic fibrous type and poorly differentiated type. In the majority of cases, the synovial sarcoma can be diagnosed through histology; however there are some cases wherein histological diagnosis may prove difficult: poorly differentiated type, unusual anatomicalpresentation (as in this case), and presentation as a metastatic tumor with unknown primary location. Inall of the aforementioned scenarios, diagnosis must be confirmed by immunohistochemistry or cytogenetic examination [5,6]. By immunohistochemistry the antibodies CD 99 and bcl2 can be detected in the tumor cells of synovial sarcoma, however the expression of these markers is not specific for synovial sarcoma. On the other hand, the antibody TLE-1 has a better sensitivity and specificity (ranging from 90% to 100%) for synovial sarcoma and can be a valuable aid in diagnosisof these types of tumors [7]. TheTLE1(transducin-like enhancer of split 1)/E (sp1) homolog, Drosophila corepressor groucho is one of four members of the TLE gene family associated with embryogenesis, hematopoiesis, and neuronal and epithelial differentiation [8]. TLE-1 is a transcriptional corepressor that binds to a number of transcription factors and plays an important role in the WNT/b-catenin signaling pathway, which is known to be associated with synovial sarcomas [9]. According to the study by Terry et al. [8] TLE-1 is an excellent discriminator of synovial sarcoma from other sarcomas that must be considered in the differential diagnosis such as Ewing’s sarcomas, malignant neoplasm of peripheral nerve sheath, malignant fibrous histiocytoma, and others. This makes the Volume 1 • Issue 1 • 1000106
5. Rong R, Doxtader EE, Tull J, de la Roza G, Zhang S (2009) Metastatic poorly differentiated monophasic synovial sarcoma to lung with unknown primary: a molecular genetic analysis. Int J Clin Exp Pathol 3: 217-221. 6. De silva MV, Mc Mahon AD, Paterson L, Reid R (2003) Identification of poorly differentiated synovial sarcoma: a comparison of clinic pathological and cytogenetic features with those of typical synovial sarcoma. Histophatology 43: 220-230. 7. Foo WC, Cruise MW, Wick MR, Hornick JL (2011) Immunohistochemical staining for TLE 1 distinguishes synovial sarcoma from histologic mimics. Am J Clin Pathol 135: 839-844. 8. Terry J, Saito T, Subramanian S, Ruttan C, Antonescu CR, et al. (2007) TLE-1 as a diagnostic immunohistochemical marker for synovial sarcoma emerging from gene expression profiling studies. Am J Surg Pathol 31: 240-246. 9. Pretto D, Barco R, Rivera J, Neel N, Gustavson, et al. (2006) The synovial sarcoma translocation protein SYT-SSX2 recruits beta-catenin to the nucleus and associates with it in an active complex. Oncogene 25: 3661-3669.
Author Affiliations
Top
Pathology Department, Hospital General de Soledad, San Luis Potosi, Mexico Surgery Department, Hospital Central “Dr. Ignacio Morones Prieto”, San Luis Potosi, Mexico 3 Dermatology Department, Temple University School of Medicine, Philadelphia, USA 1 2
Submit your next manuscript and get advantages of SciTechnol submissions
50 Journals 21 Day rapid review process 1000 Editorial team 2 Million readers More than 5000 followers Publication immediately after acceptance Quality and quick editorial, review processing
Submit your next manuscript at ● www.scitechnol.com/submission
• Page 2 of 2 •
