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), SEX . | CLINICAL FEATURES . | ASSOCIATED DIAGNOSIS . | CYTOGENETICS . | OUTCOME . |
|---|---|---|---|---|---|
| Bassichis et al. (2000) | 1, male | Masseter muscle | Synchronous AML | Not reported (NR) | Died during chemotherapy. |
| Au WY et al. (2001) | 37, male | Conductive hearing loss, infiltrative nasopharyngeal mass | Solitary site of GS | Normal | Involved field radiotherapy (IFRT) and chemotherapy. Complete remission (CR) at 3 years. |
| Nayak et al.(2001) | 24, female | Bilateral parotid and nasopharyngeal mass | Solitary site of GS | NR | patient died on 17th day of chemotherapy due to systemic infection |
| Geisse et al. (2002) | 60, male | Waldeyer’s ring lymphadenopathy | Synchronous MDS | NR | Diagnosis made on autopsy |
| Prades et al.(2002) | 20, female | Sino-nasal obstruction; right maxillary and sphenoid sinus mass | GS of the nasal cavity and paranasal sinus | t(19:1) | Allogeneic hematopoietic stem cell transplant (AHSCT) following chemotherapy; CR at 18 months |
| Ozcelik et al. (2003) | 37, male | Vocal cord paralysis, involvement of 9th, 10th, 12th cranial nerves | AML (M0) 6 months earlier - treated with chemotherapy to CR | NR | Treated with chemotherapy with partial regression of the nasopharyngeal masses; patient died on 17th day of chemotherapy due to pulmonary infection |
| Sugimoto et al.(2004) | 31, female | Nasopharynx, external acoustic meatus | AML (M2) 3 months earlier - treated with chemotherapy to CR | t(8;21)(q22;q22) | Achieved CR2 with IFRT, re-induction chemotherapy, followed by AHSCT. |
| Imamura et al.(2004) | 7, female | Waldeyer’s ring and cervical lymphadenopathy | Synchronous juvenile myelomonocytic leukemia | t(9;12) (p22;q24.1) | AHSCT following chemotherapy; CR at 3 years |
| Ferri et al. (2005) | 72, female | Right facial swelling and fever; maxillo-ethmoidal mass | AML (M0) 1 year earlier – treated with hydroxyurea. | NR | Best supportive care only; died after 10 days of hospitalization. |
| Teramoto et al.(2006) | 81, female | naso-pharyngeal mass | Developed AML (M2) 1 year later. | Complex genomic defects on cDNA microarray | Radiation therapy only for GS; chemotherapy for AML; died 6 months after diagnosis of AML. |
| Selvarajan et al. (2008) | 25, male | Dysphagia, hoarseness, facial nerve palsy | AML (M2) 4 years earlier – treated with chemotherapy to CR followed by AHSCT | t(8:21) | Treated with chemotherapy but had systemic relapse 1 year later |
| Cho et al. (2011) | 18, male | Conductive hearing loss, infiltrative nasopharyngeal mass | Synchronous AML | RUNX1-RUNX1T1 | Recurrence after 7 months of chemotherapy; achieved CR2 with re-induction chemotherapy, followed by AHSCT. |
| Mei et al. (2013) | 56, female | Left maxillary sinus | Solitary site of GS | NR | Surgical resection followed by chemotherapy; CR at 4 months |
| (Current) case | 63, female | Conductive hearing loss, infiltrative nasopharyngeal mass | Developed AML one year later. | Normal | Radiation therapy only for GS; chemotherapy for AML; CR at 18 months |
| REFERENCE (YEAR) . | AGE (YEARS), SEX . | CLINICAL FEATURES . | ASSOCIATED DIAGNOSIS . | CYTOGENETICS . | OUTCOME . |
|---|---|---|---|---|---|
| Bassichis et al. (2000) | 1, male | Masseter muscle | Synchronous AML | Not reported (NR) | Died during chemotherapy. |
| Au WY et al. (2001) | 37, male | Conductive hearing loss, infiltrative nasopharyngeal mass | Solitary site of GS | Normal | Involved field radiotherapy (IFRT) and chemotherapy. Complete remission (CR) at 3 years. |
| Nayak et al.(2001) | 24, female | Bilateral parotid and nasopharyngeal mass | Solitary site of GS | NR | patient died on 17th day of chemotherapy due to systemic infection |
| Geisse et al. (2002) | 60, male | Waldeyer’s ring lymphadenopathy | Synchronous MDS | NR | Diagnosis made on autopsy |
| Prades et al.(2002) | 20, female | Sino-nasal obstruction; right maxillary and sphenoid sinus mass | GS of the nasal cavity and paranasal sinus | t(19:1) | Allogeneic hematopoietic stem cell transplant (AHSCT) following chemotherapy; CR at 18 months |
| Ozcelik et al. (2003) | 37, male | Vocal cord paralysis, involvement of 9th, 10th, 12th cranial nerves | AML (M0) 6 months earlier - treated with chemotherapy to CR | NR | Treated with chemotherapy with partial regression of the nasopharyngeal masses; patient died on 17th day of chemotherapy due to pulmonary infection |
| Sugimoto et al.(2004) | 31, female | Nasopharynx, external acoustic meatus | AML (M2) 3 months earlier - treated with chemotherapy to CR | t(8;21)(q22;q22) | Achieved CR2 with IFRT, re-induction chemotherapy, followed by AHSCT. |
| Imamura et al.(2004) | 7, female | Waldeyer’s ring and cervical lymphadenopathy | Synchronous juvenile myelomonocytic leukemia | t(9;12) (p22;q24.1) | AHSCT following chemotherapy; CR at 3 years |
| Ferri et al. (2005) | 72, female | Right facial swelling and fever; maxillo-ethmoidal mass | AML (M0) 1 year earlier – treated with hydroxyurea. | NR | Best supportive care only; died after 10 days of hospitalization. |
| Teramoto et al.(2006) | 81, female | naso-pharyngeal mass | Developed AML (M2) 1 year later. | Complex genomic defects on cDNA microarray | Radiation therapy only for GS; chemotherapy for AML; died 6 months after diagnosis of AML. |
| Selvarajan et al. (2008) | 25, male | Dysphagia, hoarseness, facial nerve palsy | AML (M2) 4 years earlier – treated with chemotherapy to CR followed by AHSCT | t(8:21) | Treated with chemotherapy but had systemic relapse 1 year later |
| Cho et al. (2011) | 18, male | Conductive hearing loss, infiltrative nasopharyngeal mass | Synchronous AML | RUNX1-RUNX1T1 | Recurrence after 7 months of chemotherapy; achieved CR2 with re-induction chemotherapy, followed by AHSCT. |
| Mei et al. (2013) | 56, female | Left maxillary sinus | Solitary site of GS | NR | Surgical resection followed by chemotherapy; CR at 4 months |
| (Current) case | 63, female | Conductive hearing loss, infiltrative nasopharyngeal mass | Developed AML one year later. | Normal | Radiation therapy only for GS; chemotherapy for AML; CR at 18 months |
No relevant conflicts of interest to declare.
Author notes
Asterisk with author names denotes non-ASH members.
