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Granulocytic Sarcoma

Myeloid sarcoma


Presentation

  • Rarely it may present as intravascular tumor aggregates called granulocytic sarcoma which presents as intracranial hemorrhage.[ncbi.nlm.nih.gov]
Fatigue
  • Case 363 was from a 70-year-old man who had weakness and fatigue. The BM revealed AML without increased eosinophils. Conventional cytogenetic studies showed 46,XY,t(2;4)(p11.2;q12[18]/46,XY[2].[doi.org]
  • Case 221 occurred in a 5-year-old boy who had pallor, fatigue, fever, and chest and joint pain in November 2006. CT scans showed multiple lytic bone lesions in the ribs, scapula, sternum, and skull.[academic.oup.com]
Hypothermia
  • […] this TOC Section Managing antiphospholipid antibodies and antiphospholipid syndrome in children Favourable outcome in an APL patient with PLZF/RARalpha fusion gene: quantitative real-time RT-PCR confirms molecular response Recurrent hypoglycemia and hypothermia[haematologica.org]
Hemoptysis
  • (h) CD2 negative Click here to view Case 2 An 18-year-old male presented with gradually progressive cough and breathlessness for 1 month, associated with occasional hemoptysis.[jahjournal.org]
  • Adarsh Vennepureddy, Gautam Valecha, Srujitha Murukutla, Shafinaz Hussein and Terenig Terjanian, Bronchial myeloid sarcoma with concurrent Aspergillus fumigatus infection in a patient presenting with hemoptysis, Expert Review of Hematology, 10.1586/17474086.2015.1044747[doi.org]
Pleural Effusion
  • Salvatore Guastafierro, Umberto Falcone and Giuseppe Colella, Gingival swelling and pleural effusion: non‐leukemic myeloid sarcoma, European Journal of Haematology, 91, 1, (94-94), (2013).[doi.org]
Hematochezia
  • His hematochezia responded rapidly to induction chemotherapy and the patient remained in complete remission after 3-month follow-up.[ncbi.nlm.nih.gov]
Gingival Overgrowth
  • She was referred to a dentist for further evaluation, revealing generalized gingival overgrowth as well as periodontal, apical disease, and bleeding of the gums.[ncbi.nlm.nih.gov]
Murphy's Sign
  • The right upper quadrant ultrasound showed a thickened gallbladder wall with cholelithiasis, a positive sonographic Murphy sign, and marked dilatation of the common bile duct, which was up to 17 mm in diameter.[ncbi.nlm.nih.gov]
Sciatica
  • Intervertebral disc herniation can be an incidental finding and is not always the cause of sciatica.[ncbi.nlm.nih.gov]

Workup

  • It may present at any stage of AML and requires appropriate workup and treatment, though generally carries a poor prognosis.[ncbi.nlm.nih.gov]
  • MS share morphological features similar to non-Hodgkins lymphoma and both express certain markers, such as CD43 and CD45. [2] An inadequate workup may lead to confounding diagnosis.[pediatricneurosciences.com]
  • Presence of musculoskeletal complaints in leukemic patient should alert the attending trauma surgeon to carry out rigorous diagnostic workup for associated and underlying pathology.[joas.in]
  • As the initial presentation was consistent with pelvic malignancy, workup for gynecologic and urologic malignancy was done. Cervical and endometrial biopsies were negative for malignancy, and bladder wash was suspicious for malignant cells.[hematologyandoncology.net]
Periodic Lateralized Epileptiform Discharges
  • Electroencephalography demonstrated periodic lateralized epileptiform discharges. Acyclovir was initiated for herpes simplex encephalitis, however, follow-up MRI showed extension of the lesion.[ncbi.nlm.nih.gov]
Ovarian Mass
  • Here, we report the ninth case: a 27-year-old female who presented with an ovarian mass without any underlying hematologic disorder.[ncbi.nlm.nih.gov]
Pleural Effusion
  • Salvatore Guastafierro, Umberto Falcone and Giuseppe Colella, Gingival swelling and pleural effusion: non‐leukemic myeloid sarcoma, European Journal of Haematology, 91, 1, (94-94), (2013).[doi.org]

Treatment

  • This form of myeloid sarcoma is distinguished by its highly successful treatment with imatinib (the recommended treatment for FIP1L1-PDGRGA fusion gene-induced eosinophilic leukemia) rather than more aggressive and toxic therapy.[en.wikipedia.org]
  • Seven days of high-dose methyl prednisolone (HDMP) treatment (3 days 30 mg/kg/day and 4 days 20 mg/kg/day) was administered initially, and subsequently Acute Myeloid Leukaemia-Berlin Frankfurt Münster (AML-BFM) 2004 treatment protocol was continued for[ncbi.nlm.nih.gov]

Prognosis

  • In spite of the fact that t(8;21) is considered to be associated with good prognosis, patients with GS and spinal cord compression had less favorable prognosis than other AML patients with t(8;21).[ncbi.nlm.nih.gov]

Etiology

  • Further studies confirmed the etiology of the tumor. On control ultrasonography, a marked decrease in tumor size 15 days after treatment was revealed.[ncbi.nlm.nih.gov]

Epidemiology

  • To define clinical characteristics, epidemiology, pathologic findings, treatment options and outcomes in MS. We conducted a retrospective review of 23 patients diagnosed with MS at our institute over a period of 13 years (2002-2015).[ncbi.nlm.nih.gov]
  • Summary Epidemiology The exact prevalence is unknown. Clinical description The clinical presentation depends on the site of involvement.[orpha.net]
  • Presentation and outcomes among patients with isolated myeloid sarcoma: a Surveillance, Epidemiology, and End Results database analysis [published online October 9, 2014 ]. Leuk Lymphoma. doi: 10.3109/10428194.2014.963080.[doi.org]
  • Presentation and outcomes among patients with isolated myeloid sarcoma: a Surveillance, Epidemiology, and End Results database analysis. Leuk Lymphoma 56(6): 1698–1703, DOI: Lazzarotto, D Candoni, A Filì, C et al. (2017).[jbsr.be]
  • Epidemiology [ edit ] References: [1] Most frequently develop in the setting of AML but can occur with CML and MDS. Incidence of 2.5-9.1% in AML. Most common locations: skin, soft tissue, bone, periosteum, lymph nodes.[en.wikibooks.org]
Sex distribution
Age distribution

Pathophysiology

  • Pathophysiology of MS remains poorly understood, however altered homing mechanisms for myeloid lineage blasts have been implicated.[doi.org]

Prevention

  • An understanding of how extramedullary granulocytic sarcoma of the heart appears on MDCT may decrease or prevent delays in diagnosing this uncommon, although important, complication from AML.[ncbi.nlm.nih.gov]
  • The addition of immunohistochemical stains can assist in preventing such misdiagnoses and should be included in all atypical lymphoma/carcinoma cases.[doi.org]
  • In a review of 154 patients with MS, 47% were initially misdiag-nosed as soft tissue sarcomas, non-Hodgkin lympho-mas or malignant melanomas.10 In order to prevent misdiagnosis, histologic examination is obligatory.[journalofcasereport.com]

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