Lateral pharyngeal wall myeloid sarcoma as a relapse of acute biphenotypic leukemia: a case report and review of the literature

Abstract

IntroductionMyeloid sarcoma is a rare extramedullary malignant tumor composed of immature myeloid cells. The tumor can affect any part of the body. Involvement of the oral cavity and nasopharynx has been reported in 50 cases. We report a case describing myeloid sarcoma affecting the lateral pharyngeal wall.Case presentationA 31-year-old Arabian man who had acute biphenotypic leukemia treated with chemoradiation and allogeneic stem cell transplant was referred to our department with sore throat and a mass lesion in his lateral pharyngeal wall after failed antibiotic therapy. Biopsy of his lesion revealed myeloid sarcoma. He was referred to the Department of Hematology-Oncology for further evaluation that showed no other lesions.The patient was diagnosed with isolated extramedullary myeloid sarcoma of his lateral pharyngeal wall as a relapse of acute biphenotypic leukemia and managed with chemoradiation.ConclusionsMyeloid sarcoma of the pharynx is a rarely encountered malignancy in the practice of otolaryngology-head and neck surgery. It can develop de novo, but may also represent relapse of leukemia. Thus, it should be considered in the differential diagnosis of any pharyngeal lesions in patients with leukemia.

Immunohistochemical patterns in the differential diagnosis of rhinopharyngeal granulocytic sarcoma

“…Previous studies have demonstrated that immunophenotype is of utmost importance in determining the diagnosis of GS (3)(4)(5). In particular, the literature has focused on the CD13 and CD68 markers for granular monocytic and macrophagic cells, MPO and CD117 markers for myeloid differentiation, lysozyme marker for monocytic lineage, CD43 marker for myeloid cells as well as T cells and B precursors, and CD34 and terminal deoxynucleotidyl transferase (TdT) markers for immature cells (10).…”

“…The reason for this association is still unknown, but may be attributed to a pattern of graft-versus-leukemia surveillance or to the biology of high-risk AML treated with transplantation (5).…”

“…Commonly involved sites include subperiosteal bone, lymph nodes and skin. The prevalence of head and neck region involvement is 12-43% of cases (5), and orbit, skull and epidural spaces are the most frequently involved sites (7). Rare lesions have been described in the maxilla, soft palate, paranasal sinus, salivary gland, scalp and temporal bone, whereas only a few cases have been described with rhinopharyngeal involvement (4).…”

“…GS may develop de novo (solitary, primary or non-leukemic GS; 8-20%) (3), simultaneously to AML (2.5-9.1%) (4), or as a relapse of leukemia, particularly following allogeneic hematopoietic stem cell transplant (AHSCT) (4,5). The reason for this association is still unknown, but may be attributed to a pattern of graft-versus-leukemia surveillance or to the biology of high-risk AML treated with transplantation (5).…”

Mixed phenotypic acute leukemia (mixed myeloid/b-cell) with myeloid sarcoma of the thyroid gland: A rare entity with rarer asssociation - detected on fdg pet/ct

“…[ 5 ] Mixed phenotypic acute leukemia (MPAL) presenting as MS is exceedingly rare with only three cases described till date. [ 6 7 8 ] Our case of MS is unique not only in its unusual location of thyroid, but its also a rare underlying morphology of MPAL.…”