SpringerLink
Log in

Combined Modality Management of Sinonasal Anaplastic Lymphoma Kinase Negative Anaplastic Large Cell Lymphoma in a Geriatric Patient-Report of a Rare Case

  • Case Reports
  • Published:
Head and Neck Pathology Aims and scope Submit manuscript

Abstract

Sinonasal anaplastic lymphoma kinase(ALK)-negative anaplastic large cell lymphoma(ALCL) without nodal involvement is exceedingly rare and the rarity of this tumor often engenders diagnostic dilemma. It has been mostly reported in pediatric, adolescent and young adult patients, mostly of Asian origin. A 70-year-old female patient presented with a mass in the left nasal cavity causing nasal obstruction for 5 months. On clinical and radiological examination, there was a 5.7 cm mass in the left nasal cavity, completely obliterating the left ethmoid sinus. Biopsy from the nasal mass showed a poorly differentiated malignant tumour with large cells arranged in sheets. On immunohistochemistry, the tumour cells were positive for leukocyte common antigen(LCA), CD30, CD43, BCL6 and focally for CD5, TIA1, granzyme B and epithelial membrane antigen(EMA), and were negative for CD56, EBV-LMP1, CD79a, PAX5, myeloperoxidase, CD34, CD7, CD4, CD8, CD138, ALK and p63, suggestive of ALK-negative ALCL. Rest of the lymphoma work-up was essentially normal and she had stage IE disease. She was treated with prephase chemotherapy (Vincristine and Prednisolone) followed by 4 cycles of CEOP[Cyclophosphamide, Etoposide (from 2nd cycle onwards), Vincristine and Prednisolone] regimen and local radiotherapy (36 Gy/20 fractions/4 weeks) by intensity modulated radiotherapy(IMRT) technique resulting in complete clinical and radiological response. At last follow-up visit, 15 months from the initial diagnosis, she was alive and disease free. Sinonasal ALK-negative ALCL is a rare tumor which can be effectively treated with a combination of multi-agent CHOP/CHOP-like regimen and local conformal radiotherapy in geriatric patients.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Subscribe and save

Springer+ Basic
EUR 32.99 /Month
  • Get 10 units per month
  • Download Article/Chapter or Ebook
  • 1 Unit = 1 Article or 1 Chapter
  • Cancel anytime
Subscribe now

Buy Now

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

References

  1. Dolev Y, Young J, Manoukian JJ. Multifocal anaplastic large T cell lymphoma of the ethmoid sinuses, temporalis muscle and frontal lobe in a 17-year-old boy. Int J Pediatr Otorhinolaryngol Extra. 2008;3:165–9.

    Article  Google Scholar 

  2. Jamshidi P, Chen JY, Wang H, Chen CC. Anaplastic lymphoma kinase-positive large cell lymphoma of the anterior skull base: report of an unusual case and review of the literature. Surg Neurol Int. 2013;4:57.

    Article  Google Scholar 

  3. Biswas A, Devnani B, Agarwal S, Sharma MC, Sharma A. Combined modality management of sinonasal anaplastic lymphoma kinase-positive anaplastic large cell lymphoma in a young adult-report of a rare case. J Adolesc Young Adult Oncol. 2019;8(4):469–76.

    Article  Google Scholar 

  4. Zhang XM, Li YX, Wang WH, ** J, Wang SL, Liu YP, et al. Favorable outcome with doxorubicin-based chemotherapy and radiotherapy for adult patients with early stage primary systemic anaplastic large-cell lymphoma. Eur J Haematol. 2013;90(3):195–201.

    Article  CAS  Google Scholar 

  5. Giridhar P, Mallick S, Bhasker S, Pathy S, Mohanti BK, Biswas A, et al. Head and neck extra nodal NHL (HNENL)–Treatment Outcome and Pattern of failure: a single institution experience. Asian Pac J Cancer Prev. 2015;16(15):6267–72.

    Article  Google Scholar 

  6. Vega F, Lin P, Medeiros LJ. Extranodal lymphomas of the head and neck. Ann Diagn Pathol. 2005;9(6):340–50.

    Article  Google Scholar 

  7. Kinney MC, Higgins RA, Medina EA. Anaplastic large cell lymphoma: twenty five years of discovery. Arch Pathol Lab Med. 2011;135(1):19–43.

    Article  Google Scholar 

  8. Lopez-Guillermo A, Cid J, Salar A, López A, Montalbán C, Castrillo JM, et al. Peripheral T-cell lymphomas: Initial features, natural history, and prognostic factors in a series of 174 patients diagnosed according to the R.E.A.L. Classification. Ann Oncol. 1998;9(8):849–55.

    Article  CAS  Google Scholar 

  9. Morris SW, Kirstein MN, Valentine MB, Dittmer K, Shapiro DN, Look AT, et al. Fusion of a kinase gene, ALK, to a nucleolar protein gene, NPM, in non-Hodgkin’s lymphoma [letter]. Science. 1995;267:316–7.

    Article  CAS  Google Scholar 

  10. Iwahara T, Fujimoto J, Wen D, Cupples R, Bucay N, Arakawa T, et al. Molecular characterization of ALK, a receptor tyrosine kinase expressed specifically in the nervous system. Oncogene. 1997;14(4):439–49.

    Article  CAS  Google Scholar 

  11. Savage KJ, Harris NL, Vose JM, Ullrich F, Jaffe ES, Connors JM, et al. ALK- anaplastic large-cell lymphoma is clinically and immunophenotypically different from both ALK+ ALCL and peripheral T-cell lymphoma, not otherwise specified: report from the International Peripheral T-Cell Lymphoma Project. Blood. 2008;111(12):5496–504.

    Article  CAS  Google Scholar 

  12. Abbondanzo SL, Wenig BM. Non-Hodgkin’s lymphoma of the sinonasal tract. A clinicopathologic and immunophenotypic study of 120 cases. Cancer. 1995;75(6):1281–91.

    Article  CAS  Google Scholar 

  13. Chatterjee T, Singh D, Goyal H, Pautu JL, Chowdhary GS, Saxena R. Anaplastic large cell lymphoma of maxillary antrum. A case report. Otorinolaringologia. 2004;54(4):199–201.

    Google Scholar 

  14. Guida A, Meleti M, Vescovi P, Serpico R, Lucchese A, Lo Muzio L, et al. Anaplastic large cell anaplastic lymphoma kinase + non-Hodgkin lymphoma in a 10-year-old male discovered during dental visit: a case report. Head Neck Oncol. 2012;4(5):84.

    Google Scholar 

  15. Dion GR, Packer MD. Anaplastic large cell lymphoma presenting as a nasopharyngeal mass and cervical lymphadenopathy. Ear Nose Throat J. 2015;94(6):E26.

    Article  Google Scholar 

  16. Yang W, Zuo Y, Yang Y, Tao J, Hong J, Wu Z, et al. Pediatric anaplastic large cell lymphoma misdiagnosed as multiple organ abscesses: a case report and literature review. Int J Clin Exp Med. 2015;8(10):19509–16.

    PubMed  PubMed Central  Google Scholar 

  17. Kakkar A, Sharma B, Das P, Jain S, Gupta SD, Sood R. ALK-negative anaplastic large-cell lymphoma diagnosed on liver biopsy in a child presenting with nonresolving pyrexia. Indian J Med Paediatr Oncol. 2019;40(Suppl S1):169–72.

    Google Scholar 

  18. Montes-Mojarro IA, Steinhilber J, Bonzheim I, Quintanilla-Martinez L, Fend F. The pathological spectrum of systemic anaplastic large cell lymphoma (ALCL). Cancers (Basel). 2018;10(4):107. https://doi.org/10.3390/cancers10040107.

    Article  CAS  Google Scholar 

  19. Parrilla Castellar ER, Jaffe ES, Said JW, Swerdlow SH, Ketterling RP, Knudson RA, et al. ALK-negative anaplastic large cell lymphoma is a genetically heterogeneous disease with widely disparate clinical outcomes. Blood. 2014;124(9):1473–80.

    Article  Google Scholar 

  20. Onaindia A, de Villambrosía SG, Prieto-Torres L, Rodríguez-Pinilla SM, Montes-Moreno S, González-Vela C, et al. DUSP22-rearranged anaplastic lymphomas are characterized by specific morphological features and a lack of cytotoxic and JAK/STAT surrogate markers. Haematologica. 2019;104(4):e158–62.

    Article  CAS  Google Scholar 

  21. Wang X, Boddicker RL, Dasari S, Sidhu JS, Kadin ME, Macon WR, et al. Expression of p63 protein in anaplastic large cell lymphoma: implications for genetic subty**. Hum Pathol. 2017;64:19–27.

    Article  CAS  Google Scholar 

  22. Irshaid L, Xu ML. ALCL by any other name: the many facets of anaplastic large cell lymphoma. Pathology. 2020;52(1):100–10.

    Article  CAS  Google Scholar 

  23. Zinzani PL, Martelli M, Magagnoli M, Zaccaria A, Ronconi F, Cantonetti M, et al. Anaplastic large cell lymphoma Hodgkin’s-like: a randomized trial of ABVD versus MACOP-B with and without radiation therapy. Blood. 1998;92(3):790–4.

    CAS  PubMed  Google Scholar 

  24. Seidemann K, Tiemann M, Schrappe M, Yakisan E, Simonitsch I, Janka-Schaub G, et al. Short-pulse B–non-Hodgkin lymphoma–type chemotherapy is efficacious treatment for pediatric anaplastic large cell lymphoma: a report of the Berlin-Frankfurt-Mu¨nster Group Trial NHL-BFM 90. Blood. 2001;97(12):3699–706.

    Article  CAS  Google Scholar 

  25. Brugieres L, Le Deley MC, Rosolen A, Williams D, Horibe K, Wrobel G, et al. Impact of the methotrexate administration dose on the need for intrathecal treatment in children and adolescents with anaplastic large-cell lymphoma: results of a randomized trial of the EICNHL Group. J Clin Oncol. 2009;27(6):897–903.

    Article  CAS  Google Scholar 

  26. Le Deley MC, Rosolen A, Williams DM, Horibe K, Wrobel G, Attarbaschi A, et al. Vinblastine in children and adolescents with high-risk anaplastic large-cell lymphoma: results of the randomized ALCL99-Vinblastine trial. J Clin Oncol. 2010;28(25):3987–93.

    Article  Google Scholar 

  27. Lowe EJ, Sposto R, Perkins SL, Gross TG, Finlay J, Zwick D, et al. Intensive Chemotherapy for systemic anaplastic large cell lymphoma in children and adolescents: final results of Children’s Cancer Group Study 5941. Pediatr Blood Cancer. 2009;52(3):335–9.

    Article  Google Scholar 

Download references

Funding

This work received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

Author information

Authors and Affiliations

  1. Departments of Radiotherapy & Oncology, All India Institute of Medical Sciences, New Delhi, India

    Ahitagni Biswas, Sorun Shishak & Swarnaditya Roy

  2. Department of Pathology, All India Institute of Medical Sciences, New Delhi, India

    Aanchal Kakkar

Authors
  1. Ahitagni Biswas
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Sorun Shishak
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Swarnaditya Roy
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Aanchal Kakkar
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

All authors contributed to the study conception and design. AK was responsible for the diagnosis of the tumor based on examination of the histopathology specimen and relevant immunohistochemical studies. AB was responsible for the treatment plan and clinical care of the patient during administration of chemotherapy and radiotherapy. SS assisted in patient care during chemotherapy and SR assisted in patient care during radiotherapy planning and delivery. The pertinent medical literature was reviewed by AB and AK. The first draft of the manuscript was written by AB and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

Corresponding author

Correspondence to Ahitagni Biswas.

Ethics declarations

Conflict of interest

The authors declare that there is no conflict of interest.

Informed Consent

The patient gave written informed consent before the administration of chemotherapy and radiotherapy. Informed consent for patient information and images to be published was also provided by the patient.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Biswas, A., Shishak, S., Roy, S. et al. Combined Modality Management of Sinonasal Anaplastic Lymphoma Kinase Negative Anaplastic Large Cell Lymphoma in a Geriatric Patient-Report of a Rare Case. Head and Neck Pathol 15, 1335–1344 (2021). https://doi.org/10.1007/s12105-020-01276-3

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12105-020-01276-3

Keywords

Search

Navigation