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The diagnosis and management of primary and iatrogenic soft tissue sarcomas of the sella

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Abstract

Purpose

Soft tissue sarcoma (STS) of the sella is exceptionally rare. We conducted a case series, literature review, and nationwide analysis of primary and iatrogenic (radiation-associated) STS of the sella to define the clinical course of this entity.

Methods

This study employed a multi-institutional retrospective case review, literature review, and nationwide analysis using the National Cancer Database (NCDB).

Results

We report five patients who were diagnosed at three institutions with malignant STS of the sella. All patients presented with symptoms related to mass effect in the sellar region. All tumors extended to the suprasellar space, with the majority displaying extension into the cavernous sinus. All patients underwent an operation via a transsphenoidal approach with a goal of maximal safe tumor resection in four patients and biopsy for 1 patient. Histopathologic evaluation demonstrated STS in all patients. Post-operative adjuvant radiotherapy and chemotherapy were given to 2 and 1 out of 4 patients with known post-operative clinical course, respectively. The 1-year and 5-year overall survival rates were 100% (5/5) and 25% (1/4). Twenty-two additional reports of primary, non-iatrogenic STS of the sella were identified in the literature. Including the three cases from our series, treatment included resection in all cases, and adjuvant radiotherapy and chemotherapy were utilized in 50% (12/24) and 17% (4/24) of cases, respectively. The national prevalence of malignant STS is estimated to be 0.01% among all pituitary and sellar tumors within the NCDB.

Conclusions

We report the prevalence and survival rates of STS of the sella. Multimodal therapy, including maximal safe resection, chemotherapy, and radiotherapy are necessary to optimize outcomes for this uncommon pathology.

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References

  1. Lopes MB, Lanzino G, Cloft HJ, Winston DC, Vance ML, Laws ER Jr (1998) Primary fibrosarcoma of the sella unrelated to previous radiation therapy. Mod Pathol 11(6):579–584

    CAS  PubMed  Google Scholar 

  2. Manoranjan B, Syro LV, Scheithauer BW, Ortiz LD, Horvath E, Salehi F, Kovacs K, Cusimano MD (2011) Undifferentiated sarcoma of the sellar region. Endocr Pathol 22(3):159–164. https://doi.org/10.1007/s12022-011-9166-7

    Article  PubMed  Google Scholar 

  3. Sareen P, Chhabra L, Trivedi N (2013) Primary undifferentiated spindle-cell sarcoma of sella turcica: successful treatment with adjuvant temozolomide. BMJ Case Rep. https://doi.org/10.1136/bcr-2013-009934

    Article  PubMed  PubMed Central  Google Scholar 

  4. Abele TA, Yetkin ZF, Raisanen JM, Mickey BE, Mendelsohn DB (2012) Non-pituitary origin sellar tumours mimicking pituitary macroadenomas. Clin Radiol 67(8):821–827. https://doi.org/10.1016/j.crad.2012.01.001

    Article  CAS  PubMed  Google Scholar 

  5. Boffa DJ, Rosen JE, Mallin K, Loomis A, Gay G, Palis B, Thoburn K, Gress D, McKellar DP, Shulman LN, Facktor MA, Winchester DP (2017) Using the national cancer database for outcomes research: a review. JAMA Oncol 3(12):1722–1728. https://doi.org/10.1001/jamaoncol.2016.6905

    Article  PubMed  Google Scholar 

  6. Zhong J, Li ST, Yao XH, Jin B, Wan L (2007) An intrasellar rhabdomyosarcoma misdiagnosed as pituitary adenoma. Surg Neurol 68(Suppl 2):S29–S33. https://doi.org/10.1016/j.surneu.2007.01.079. (discussion S33)

    Article  PubMed  Google Scholar 

  7. Lleva RR, Inzucchi SE (2011) Diagnosis and management of pituitary adenomas. Curr Opin Oncol 23(1):53–60. https://doi.org/10.1097/CCO.0b013e328341000f

    Article  PubMed  Google Scholar 

  8. Furtado SV, Ghosal N, Venkatesh PK, Gupta K, Hegde AS (2010) Diagnostic and clinical implications of pituicytoma. J Clin Neurosci 17(7):938–943. https://doi.org/10.1016/j.jocn.2009.09.047

    Article  PubMed  Google Scholar 

  9. Hammoud DA, Munter FM, Brat DJ, Pomper MG (2010) Magnetic resonance imaging features of pituicytomas: analysis of 10 cases. J Comput Assist Tomogr 34(5):757–761. https://doi.org/10.1097/RCT.0b013e3181e289c0

    Article  PubMed  Google Scholar 

  10. Kurosaki M, Kambe A, Ishibashi M, Watanabe T, Horie Y (2014) Case report of sarcoma of the sella caused by postoperative radiotherapy for a prolactin-producing pituitary adenoma. Brain Tumor Pathol 31(3):187–191. https://doi.org/10.1007/s10014-014-0175-3

    Article  PubMed  Google Scholar 

  11. Chowdhary A, Spence AM, Sales L, Rostomily RC, Rockhill JK, Silbergeld DL (2012) Radiation associated tumors following therapeutic cranial radiation. Surg Neurol Int 3:48. https://doi.org/10.4103/2152-7806.96068

    Article  PubMed  PubMed Central  Google Scholar 

  12. Berkmann S, Tolnay M, Hanggi D, Ghaffari A, Gratzl O (2010) Sarcoma of the sella after radiotherapy for pituitary adenoma. Acta Neurochir (Wien) 152(10):1725–1735. https://doi.org/10.1007/s00701-010-0694-6

    Article  Google Scholar 

  13. Wu-Chen WY, Jacobs DA, Volpe NJ, Dalmau JO, Moster ML (2009) Intracranial malignancies occurring more than 20 years after radiation therapy for pituitary adenoma. J Neuroophthalmol 29(4):289–295. https://doi.org/10.1097/WNO.0b013e3181b4a1be

    Article  PubMed  Google Scholar 

  14. Minniti G, Traish D, Ashley S, Gonsalves A, Brada M (2005) Risk of second brain tumor after conservative surgery and radiotherapy for pituitary adenoma: update after an additional 10 years. J Clin Endocrinol Metab 90(2):800–804. https://doi.org/10.1210/jc.2004-1152

    Article  CAS  PubMed  Google Scholar 

  15. Terry RD, Hyams VJ, Davidoff LM (1959) Combined nonmetastasizing fibrosarcoma and chromophobe tumor of the pituitary. Cancer 12(4):791–798. https://doi.org/10.1002/1097-0142(195907/08)12:4<791:aid-cncr2820120424>3.0.co;2-b

    Article  CAS  PubMed  Google Scholar 

  16. Goldberg MB, Sheline GE, Malamud N (1963) Malignant intracranial neoplasms, following radiation therapy for acromegaly. Radiology 80:465–470. https://doi.org/10.1148/80.3.465

    Article  CAS  PubMed  Google Scholar 

  17. Dracham CB, Shankar A, Madan R (2018) Radiation induced secondary malignancies: a review article. Radiat Oncol J 36(2):85–94. https://doi.org/10.3857/roj.2018.00290

    Article  PubMed  PubMed Central  Google Scholar 

  18. Coppeto JR, Roberts M (1979) Fibrosarcoma after proton-beam pituitary ablation. Arch Neurol 36(6):380–381. https://doi.org/10.1001/archneur.1979.00500420090014

    Article  CAS  PubMed  Google Scholar 

  19. Sasagawa Y, Tachibana O, Iizuka H (2013) Undifferentiated sarcoma of the cavernous sinus after gamma knife radiosurgery for pituitary adenoma. J Clin Neurosci 20(8):1152–1154. https://doi.org/10.1016/j.jocn.2012.09.032

    Article  PubMed  Google Scholar 

  20. Pendharkar AV, Lin CY, Born DE, Hoffman AR, Dodd RL (2019) Granular cell pituitary tumor in a patient with multiple endocrine neoplasia-1. Cureus 11(4):e4541. https://doi.org/10.7759/cureus.4541

    Article  PubMed  PubMed Central  Google Scholar 

  21. Giantini Larsen AM, Cote DJ, Zaidi HA, Bi WL, Schmitt PJ, Iorgulescu JB, Miller MB, Smith TR, Lopes MB, Jane JA, Laws ER (2018) Spindle cell oncocytoma of the pituitary gland. J Neurosurg 131(2):517–525. https://doi.org/10.3171/2018.4.JNS18211

    Article  PubMed  Google Scholar 

  22. Campana D, Nori F, Piscitelli L, Morselli-Labate AM, Pezzilli R, Corinaldesi R, Tomassetti P (2007) Chromogranin A: is it a useful marker of neuroendocrine tumors? J Clin Oncol 25(15):1967–1973. https://doi.org/10.1200/JCO.2006.10.1535

    Article  CAS  PubMed  Google Scholar 

  23. Italiano A, Di Mauro I, Rapp J, Pierron G, Auger N, Alberti L, Chibon F, Escande F, Voegeli AC, Ghnassia JP, Keslair F, Lae M, Ranchere-Vince D, Terrier P, Baffert S, Coindre JM, Pedeutour F (2016) Clinical effect of molecular methods in sarcoma diagnosis (GENSARC): a prospective, multicentre, observational study. Lancet Oncol 17(4):532–538. https://doi.org/10.1016/S1470-2045(15)00583-5

    Article  CAS  PubMed  Google Scholar 

  24. Lindsay T, Movva S (2018) Role of molecular profiling in soft tissue sarcoma. J Natl Compr Canc Netw 16(5):564–571. https://doi.org/10.6004/jnccn.2018.7015

    Article  CAS  PubMed  Google Scholar 

  25. NCCN Clinical Practice Guidelines in Oncology. Soft Tissue Sarcoma, Version 9 National Comprehensive Cancer Network, Inc Version 2.2008 (2019).

  26. Treutwein M, Steger F, Loeschel R, Koelbl O, Dobler B (2020) The influence of radiotherapy techniques on the plan quality and on the risk of secondary tumors in patients with pituitary adenoma. BMC Cancer 20(1):88. https://doi.org/10.1186/s12885-020-6535-y

    Article  PubMed  PubMed Central  Google Scholar 

  27. Hara H, Kawamoto T, Fukase N, Kawakami Y, Takemori T, Fujiwara S, Kitayama K, Nishida K, Kuroda R, Akisue T (2019) Gemcitabine and docetaxel combination chemotherapy for advanced bone and soft tissue sarcomas: protocol for an open-label, non-randomised, Phase 2 study. BMC Cancer 19(1):725. https://doi.org/10.1186/s12885-019-5923-7

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  28. Seddon B, Strauss SJ, Whelan J, Leahy M, Woll PJ, Cowie F, Rothermundt C, Wood Z, Benson C, Ali N, Marples M, Veal GJ, Jamieson D, Kuver K, Tirabosco R, Forsyth S, Nash S, Dehbi HM, Beare S (2017) Gemcitabine and docetaxel versus doxorubicin as first-line treatment in previously untreated advanced unresectable or metastatic soft-tissue sarcomas (GeDDiS): a randomised controlled phase 3 trial. Lancet Oncol 18(10):1397–1410. https://doi.org/10.1016/S1470-2045(17)30622-8

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  29. Mir O, Brodowicz T, Italiano A, Wallet J, Blay JY, Bertucci F, Chevreau C, Piperno-Neumann S, Bompas E, Salas S, Perrin C, Delcambre C, Liegl-Atzwanger B, Toulmonde M, Dumont S, Ray-Coquard I, Clisant S, Taieb S, Guillemet C, Rios M, Collard O, Bozec L, Cupissol D, Saada-Bouzid E, Lemaignan C, Eisterer W, Isambert N, Chaigneau L, Cesne AL, Penel N (2016) Safety and efficacy of regorafenib in patients with advanced soft tissue sarcoma (REGOSARC): a randomised, double-blind, placebo-controlled, phase 2 trial. Lancet Oncol 17(12):1732–1742. https://doi.org/10.1016/S1470-2045(16)30507-1

    Article  CAS  PubMed  Google Scholar 

  30. Willis RA (1938) Pathological study of tumors of pituitary region. Med J Austr 1:287–291

    Article  Google Scholar 

  31. Anderson WR, Cameron JD, Tsai SH (1980) Primary intracranial leiomyosarcoma. Case report with ultrastructural study. J Neurosurg 53(3):401–405. https://doi.org/10.3171/jns.1980.53.3.0401

    Article  CAS  PubMed  Google Scholar 

  32. Nagasaka T, Nakashima N, Furui A, Wakabayashi T, Yoshida J (1998) Sarcomatous transformation of pituitary adenoma after bromocriptine therapy. Hum Pathol 29(2):190–193

    Article  CAS  Google Scholar 

  33. Allan CA, Kaltsas G, Evanson J, Geddes J, Lowe DG, Plowman PN, Grossman AB (2001) Pituitary chondrosarcoma: an unusual cause of a sellar mass presenting as a pituitary adenoma. J Clin Endocrinol Metab 86(1):386–391. https://doi.org/10.1210/jcem.86.1.7111

    Article  CAS  PubMed  Google Scholar 

  34. Arita K, Sugiyama K, Tominaga A, Yamasaki F (2001) Intrasellar rhabdomyosarcoma: case report. Neurosurgery 48(3):677–680

    Article  CAS  Google Scholar 

  35. Alpert TE, Hahn SS, Chung CT, Bogart JA, Hodge CJ, Montgomery C (2002) Successful treatment of spindle cell sarcoma of the sella turcica. Case report J Neurosurg 97(5 Suppl):438–440. https://doi.org/10.3171/jns.2002.97.supplement

    Article  PubMed  Google Scholar 

  36. Inenaga C, Morii K, Tamura T, Tanaka R, Takahashi H (2003) Mesenchymal chondrosarcoma of the sellar region. Acta Neurochir (Wien) 145(7):593–597. https://doi.org/10.1007/s00701-003-0059-5. (discussion 597)

    Article  CAS  Google Scholar 

  37. Moro M, Giannini C, Scheithauer BW, Lloyd RV, Restall P, Eagleton C, Law AJ, Kovacs K (2004) Combined sellar fibrosarcoma and prolactinoma with neuronal metaplasia: report of a case unassociated with radiotherapy. Endocr Pathol 15(2):149–158

    Article  Google Scholar 

  38. Massier A, Scheithauer BW, Taylor HC, Clark C, Llerena L (2007) Sclerosing epithelioid fibrosarcoma of the pituitary. Endocr Pathol 18(4):233–238. https://doi.org/10.1007/s12022-007-9010-2

    Article  PubMed  Google Scholar 

  39. Scheithauer BW, Silva AI, Kattner K, Seibly J, Oliveira AM, Kovacs K (2007) Synovial sarcoma of the sellar region. Neuro Oncol 9(4):454–459. https://doi.org/10.1215/15228517-2007-029

    Article  PubMed  PubMed Central  Google Scholar 

  40. Li ZJ, Sun P, Guo Y, Wang RZ (2010) Primary pituitary fibrosarcoma presenting with multiple metastases: a case report and literature review. Neurol India 58(2):316–318. https://doi.org/10.4103/0028-3886.63792

    Article  PubMed  Google Scholar 

  41. Smolle E, Mokry M, Haybaeck J (2015) Rare case of a primary intracranial chondrosarcoma. Anticancer Res 35(2):875–880

    PubMed  Google Scholar 

  42. Ganaha T, Inamasu J, Oheda M, Hasegawa M, Hirose Y, Abe M (2016) Subarachnoid hemorrhage caused by an undifferentiated sarcoma of the sellar region. Surg Neurol Int 7(Suppl 16):S459–462. https://doi.org/10.4103/2152-7806.185775

    Article  PubMed  PubMed Central  Google Scholar 

  43. Duncan VE, Nabors LB, Warren PP, Conry RM, Willey CD, Perry A, Riley KO, Hackney JR (2016) Primary sellar rhabdomyosarcoma arising in association with a pituitary adenoma. Int J Surg Pathol 24(8):753–756. https://doi.org/10.1177/1066896916658955

    Article  PubMed  Google Scholar 

  44. Bayoumi AB, Chen JX, Swiatek PR, Laviv Y, Kasper EM (2017) Primary Pituitary Fibrosarcoma with Previous Adenoma. World Neurosurg 105(1032):e1037-1032 e1011. https://doi.org/10.1016/j.wneu.2017.05.096

    Article  Google Scholar 

  45. Dutta G, Singh D, Singh H, Srivastava AK, Jagetia A, Sachdeva D (2018) Pituitary fossa chondrosarcoma: an unusual cause of a sellar suprasellar mass masquerading as pituitary adenoma. Surg Neurol Int 9:76. https://doi.org/10.4103/sni.sni_455_17

    Article  PubMed  PubMed Central  Google Scholar 

  46. Cao J, Li G, Sun Y, Hong X, Huang H (2018) Sellar chondrosarcoma presenting with amenorrhea: a case report. Medicine (Baltimore) 97(27):e11274. https://doi.org/10.1097/MD.0000000000011274

    Article  Google Scholar 

  47. Zhang Y, Huang J, Zhang C, Jiang C, Ding C, Lin Y, Wu X, Wang C, Kang D, Lin Z (2019) An extended endoscopic endonasal approach for sellar area chondrosarcoma: a case report and literature review. World Neurosurg 127:469–477. https://doi.org/10.1016/j.wneu.2019.04.075

    Article  PubMed  Google Scholar 

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Funding

JBI acknowledges support from the NIH (5T32HL007627). J.D.B has positions and equity in CITC Ltd and Avidea Technologies and is on the Scientific Board of Advisors for POCKiT Diagnostics.

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Author notes

  1. Edward R. Laws and Timothy R. Smith have contributed equally.

Authors and Affiliations

  1. Department of Neurosurgery, Brigham and Women’s Hospital, Harvard Medical School, 75 Francis Street, Boston, MA, 02115, USA

    Saksham Gupta, Samantha Hoffman, Joshua D. Bernstock, Melissa Chua, David J. Segar, Edward R. Laws & Timothy R. Smith

  2. Computational Neurosciences Outcome Center, Department of Neurosurgery, Brigham and Women’s Hospital, Boston, MA, 02115, USA

    Saksham Gupta, J. Bryan Iorgulescu & Timothy R. Smith

  3. Department of Pathology, Brigham and Women’s Hospital, Boston, MA, 02115, USA

    J. Bryan Iorgulescu

  4. Department of Medical Oncology, Dana Farber Cancer Institute, Boston, MA, 02115, USA

    J. Bryan Iorgulescu

  5. Department of Neurosurgery, University of North Carolina Medical Center, Chapel Hill, NC, 27599, USA

    Michael Catalino

  6. Department of Orthopedic Surgery, University of Utah School of Medicine, Salt Lake City, UT, USA

    Luis Bradley Fandino

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  1. Saksham Gupta
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Contributions

SG, JBI, LBF, and TRS were responsible for the initial conceptualization of this manuscript. SG, JBI, SH, and LBF were responsible for chart review. SG, JBI, MC, JDB, MC, DJS, ERL, and TRS were responsible for data analysis and interpretation. SG, JDB, MC, DJS, and LBF were responsible for drafting the manuscript. JBI, MC, ERL, and TRS were responsible for critical review of the manuscript. All authors contributed to final review of the manuscript and approved it.

Corresponding author

Correspondence to Timothy R. Smith.

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Conflict of interest

All authors declare no conflicts of interest or competing interests.

Ethical approval

This study was conducted under IRB approval. All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

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The requirement for informed consent for retrospective chart reviews was waived by our IRB.

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11102_2020_1062_MOESM1_ESM.tif

Supplementary Figure 1. Representative Histology of a Primary Unclassified Sarcoma of the Sella. Representative histology from a case of a primary unclassifed sarcoma of the sella (Case 4, hematoxylin and eosin 200× magnification) demonstrating poorly-differentiated spindle cell morphology with up to 21 mitoses per 10 HPF, but no further distinguishing histological features. Immunohistochemistry (negative for GFAP, OLIG2, desmin, chromogranin, SMA, CD34, pan-keratin, S100, and EMA), targeted sequencing (of 41 cancer-related genes), and karyotyping (no cytogenetic abnormalities) were additionally equivocal (TIF 13805 kb)

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Gupta, S., Iorgulescu, J.B., Hoffman, S. et al. The diagnosis and management of primary and iatrogenic soft tissue sarcomas of the sella. Pituitary 23, 558–572 (2020). https://doi.org/10.1007/s11102-020-01062-y

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