Granulozytisches Tonsillensarkom 
bei myelodysplastischem Syndrom

Geisse, M.; Mall, G; Fritze, Danielle; Gartenschläger, M

doi:10.1055/s-2002-36114

Cited by 6 publications

(1 citation statement)

References 12 publications

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“…18 Utilizing a MEDLINE ® /PubMed ® search, with the key words "granulocytic/myeloid sarcoma," "chloroma," "nasopharynx," and "acute myeloid leukemia" and restricting our search to the English language reports between the years 2000-2013, we identified 13 additional patients between the ages of 1 to 81 years (median age of 37 years) with GS involving the nasopharynx (Table 1). 4,[19][20][21][22][23][24][25][26][27][28][29][30] The male to female ratio within the group was 1:1. Sinonasal congestion or hearing loss was the reason why most patients sought medical attention.…”

Section: Discussion and Literature Reviewmentioning

confidence: 99%

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

Chuda

Vishnu

Hwang

et al. 2013

Blood

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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.

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Section: Discussion and Literature Reviewmentioning

confidence: 99%

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

Chuda

Vishnu

Hwang

et al. 2013

Blood

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Post-tonsillectomy pulmonary complication in a patient with tonsillar myeloid sarcoma

Cheng

Ueng

et al. 2011

Int J Hematol

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Myeloid sarcoma in a patient with myelodysplastic syndrome (MDS) manifesting as a non-healing tonsillar ulcer is an extremely rare occurrence. We report the case of a 57-year-old male smoker with a non-healing tonsillar ulcer who underwent tonsillectomy to rule out tonsillar carcinoma after failed antibiotic therapy. On postoperative day 2, he presented with a temperature of 40°C and white blood cell count of 34700/μL. Antibiotic therapy was begun; however, he died 1 day later due to pulmonary infection and septic shock. Though extremely rare, tonsillar involvement of MDS should be considered in the differential diagnosis of a non-healing tonsillar lesion. When definitive diagnosis requires a tissue sample, punch biopsy may be preferable to tonsillectomy in a patient who may be immunocompromised, and appropriate prophylactic antibiotics should be administered.

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Granulocytic Sarcoma Presenting with Severe Adenopathy (Cervical Lymph Nodes, Tonsils, and Adenoids) in a Child with Juvenile Myelomonocytic Leukemia and Successful Treatment with Allogeneic Bone Marrow Transplantation

Imamura

Matsuo

Yoshihara

et al. 2004

International Journal of Hematology

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The occurrence of adenopathy in patients with myelodysplastic syndrome-associated extramedullary myeloid cell tumors has rarely been reported. We describe a 7-year-old girl with juvenile myelomonocytic leukemia who showed the novel chromosomal abnormality t(9;12)(p22;q24.1) and who developed severe adenopathy of the cervical lymph nodes, tonsils, and adenoids that was manifested as granulocytic sarcoma. Following chemotherapy, the patient underwent a conditioning regimen of busulfan, cyclophosphamide, and total body irradiation followed by successful allogeneic bone marrow transplantation from her single HLA locus-mismatched mother at 6 months after her diagnosis. The patient continues to be well and in remission 3 years after stem cell transplantation.

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Granulozytisches Tonsillensarkom bei myelodysplastischem Syndrom

Abstract: Granulocytic sarcoma is rarely associated with myelodysplastic syndrome. Deficiency in the immune system may facilitate the occurrence of miliary tuberculosis.

Cited by 6 publications

References 12 publications

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

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

Post-tonsillectomy pulmonary complication in a patient with tonsillar myeloid sarcoma

Granulocytic Sarcoma Presenting with Severe Adenopathy (Cervical Lymph Nodes, Tonsils, and Adenoids) in a Child with Juvenile Myelomonocytic Leukemia and Successful Treatment with Allogeneic Bone Marrow Transplantation

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