Isolated Granulocytic Sarcoma Of The Nasopharynx: A Case Report and Review Of The Literature

Abstract

Granulocytic sarcoma (GS) is a rare extra medullary manifestation of acute myeloid leukemia (AML). It may also represent blastic transformation of myelodysplastic syndromes or myeloproliferative neoplasms. Although usually seen in the context of advanced and poorly controlled disease, it may also present as the first manifestation of illness, without concurrent bone marrow or blood involvement. In the medical literature chloroma and GS are terms that have been used interchangeably with myeloid sarcoma. GS usually manifest as soft tissue or bony masses in several extra cranial sites such as bone, periosteum and lymph nodes; involvement of the head and neck region is uncommon. Herein, we report a case of a 63 yearr-old woman with insidious onset of progressive nasal congestion and diminished hearing who was diagnosed with an isolated GS of the nasopharynx. With involved field radiotherapy she achieved a complete remission of 12 months duration before being diagnosed with overt AML. Following induction and consolidation chemotherapy she has remained disease free for now greater than 18 months. Through a MEDLINE®/PubMed® search we identified an additional 13 cases of nasopharyngeal GC. The median age was 37 years (range, 1 to 81 years). The cases were equally distributed among the sexes. The most common presenting symptoms were conductive hearing loss and sino-nasal congestion. Isolated GC was identified in 6 cases and the median time from diagnosis of GS to AML was 12 months (range, 3 to 48 months). Treatment varied, but responses were seen in all patients who received chemotherapy with or without radiotherapy.REFERENCE (YEAR)AGE (YEARS), SEXCLINICAL FEATURESASSOCIATED DIAGNOSISCYTOGENETICSOUTCOMEBassichis et al. (2000)1, maleMasseter muscleSynchronous AMLNot reported (NR)Died during chemotherapy.Au WY et al. (2001)37, maleConductive hearing loss, infiltrative nasopharyngeal massSolitary site of GSNormalInvolved field radiotherapy (IFRT) and chemotherapy. Complete remission (CR) at 3 years.Nayak et al.(2001)24, femaleBilateral parotid and nasopharyngeal massSolitary site of GSNRpatient died on 17th day of chemotherapy due to systemic infectionGeisse et al. (2002)60, maleWaldeyer’s ring lymphadenopathySynchronous MDSNRDiagnosis made on autopsyPrades et al.(2002)20, femaleSino-nasal obstruction; right maxillary and sphenoid sinus massGS of the nasal cavity and paranasal sinust(19:1)Allogeneic hematopoietic stem cell transplant (AHSCT) following chemotherapy; CR at 18 monthsOzcelik et al. (2003)37, maleVocal cord paralysis, involvement of 9th, 10th, 12th cranial nervesAML (M0) 6 months earlier - treated with chemotherapy to CRNRTreated with chemotherapy with partial regression of the nasopharyngeal masses; patient died on 17th day of chemotherapy due to pulmonary infectionSugimoto et al.(2004)31, femaleNasopharynx, external acoustic meatusAML (M2) 3 months earlier - treated with chemotherapy to CRt(8;21)(q22;q22)Achieved CR2 with IFRT, re-induction chemotherapy, followed by AHSCT.Imamura et al.(2004)7, femaleWaldeyer’s ring and cervical lymphadenopathySynchronous juvenile myelomonocytic leukemiat(9;12) (p22;q24.1)AHSCT following chemotherapy; CR at 3 yearsFerri et al. (2005)72, femaleRight facial swelling and fever; maxillo-ethmoidal massAML (M0) 1 year earlier – treated with hydroxyurea.NRBest supportive care only; died after 10 days of hospitalization.Teramoto et al.(2006)81, femalenaso-pharyngeal massDeveloped AML (M2) 1 year later.Complex genomic defects on cDNA microarrayRadiation therapy only for GS; chemotherapy for AML; died 6 months after diagnosis of AML.Selvarajan et al. (2008)25, maleDysphagia, hoarseness, facial nerve palsyAML (M2) 4 years earlier – treated with chemotherapy to CR followed by AHSCTt(8:21)Treated with chemotherapy but had systemic relapse 1 year laterCho et al. (2011)18, maleConductive hearing loss, infiltrative nasopharyngeal massSynchronous AMLRUNX1-RUNX1T1Recurrence after 7 months of chemotherapy; achieved CR2 with re-induction chemotherapy, followed by AHSCT.Mei et al. (2013)56, femaleLeft maxillary sinusSolitary site of GSNRSurgical resection followed by chemotherapy; CR at 4 months(Current) case63, femaleConductive hearing loss, infiltrative nasopharyngeal massDeveloped AML one year later.NormalRadiation therapy only for GS; chemotherapy for AML; CR at 18 months Disclosures: No relevant conflicts of interest to declare.