Isolated granulocytic sarcoma of the pancreas: A tricky diagnostic for primary pancreatic extramedullary acute myeloid leukemia
© Messager et al; licensee BioMed Central Ltd. 2012
Received: 1 November 2011
Accepted: 16 January 2012
Published: 16 January 2012
We report two clinical cases of primary granulocytic sarcoma of the pancreas that were diagnosed on the surgical specimen. Atypical clinical and morphological presentations may have lead to pretherapeutic biopsies of the pancreatic mass in order to indicate primary chemotherapy. Literature review of this rare clinical presentation may help physicians to anticipate diagnostic and therapeutic strategies.
KeywordsGranulocytic sarcoma Chloroma Myeloid tumor Pancreas.
Granulocytic sarcoma (GS) is an extramedullary solid tumor mass composed of immature myeloid cells . GS is a rare manifestation of acute myeloid leukemia (AML) usually arising during or after the course of the disease, in up to 8% of patients in autopsy studies . Occasionally, it can be the first and the only manifestation of AML, leading to diagnostic challenges. We report two exceptional cases of isolated pancreatic GS to focus physicians' attention to specific diagnostic and therapeutic strategies for a solid pancreatic mass.
Clinical characteristics, treatment and outcomes of literature reports of pancreatic granulocytic sarcomas
Author/Year of report
King et al./1987
Radiotherapy + CT (Daunorubicin, Cytarabine, Thioguanine)
Moreau et al./1996
Duodenopancreatectomy + CT (Idarubin, Cytarabine)
CR after 2 years follow-up
Marcos et al./1997
Died after initial MRI
Ravandi - Kashani et al./1999
CT (Idarubicin, Cytarabine, All-trans retinoic acid)
CR, (follow-up unknown)
CT (Idarubicin, Cytarabine, Lisofylline)
Servin-Abad et al./2003
CT (Unknown regimen)
CR, died of stroke
Breccia et al./2003
CT (Cytosine, Arabinoside, Idarubicin)+ BM allogarft
CR at 49 months from graft
Schäfer et al./2008
CT (Etoposide, Cytarabine, reduced dose Mitoxantrone)
Recurrence (7 months), died
Duodenopancreatectomy + CT (Cytarabine based regimen)
CR, (follow-up unknown)
Li et al./2011
Distal pancreatectomy + splenectomy, patient refused adjuvant CT
Recurrence (2 months), died 3 months after surgery
CT after duodenopancreatectomy
(Cisplatin, Aracytine, Dexamethasone)
Early recurrence, died
CT (Aracytine based regimen)
Recurrence (8 months), alive after BM transplantation (22 months follow-up)
GS can occur in patients of all ages with a focus on male patients (male:female ratio 1.2:1) during the last decades of life (median age is 56 years, range: 1 month - 89 years) [7, 16]. Even if the overall prognosis of AML is favorable, the association with GS makes worsens the prognosis because only 24% of patients with GS will be alive 2 years after the initial diagnosis, with an overall median survival of 7 to 20 months [3, 17].
Clinical behavior and response to therapy were not influenced by any of the following factors: age, sex, anatomic site, de novo presentation, histotype, phenotype or cytogenetic findings . It remains uncertain what constitutes the best treatment in GS-associated AML patients . However, high-dose chemotherapy and stem cell transplantation may benefit these patients, whereas radiation therapy or surgical resection have been found to be less effective .
These observations show that clinicians should think about pancreatic GS when the pancreatic mass develops during or after AML. However, in the cases reported here in which GS was the first and the only manifestation of AML, diagnosis is challenging. Because surgery is not required and may probably worsen the prognosis due to the delayed administration of induction chemotherapy, all efforts should be made to obtain pretherapeutic biopsies for a pancreatic mass, especially if all of the biological and morphological exam results are not typical and in agreement. The negative value of CA19.9 as well as the young age of our patients may have been warnings that indicate the value of EUS cytological examination for detecting differential diagnoses of pancreatic adenocarcinoma.
A positive diagnosis of GS is sometimes challenging and requires expert pathologists. Histological observation reveals myeloblats, promyelocytes and sometimes neutrophils. The definitive diagnosis of GS requires positive immunostaining for at least one of the myeloid-associated antigens (in decreasing frequency: CD68, MPO, CD43, CD45, CD117, CD99, CD33, CD34, CD13) associated with negative immunostaining for the lymphoid lineages (CD3 for T-cells and CD20 for B-cells) [1, 12]. Major differential diagnoses are Hodgkin lymphoma, Burkitt lymphoma, large-cell lymphoma, and small round cell tumours. When a histological diagnosis of GS is made, bone marrow sampling is mandatory to assess the absence of AML.
The risk of metachronous AML occurrence in non-leukemic patients with GS is very high, with a median delay of 5 months; most patients will develop AML within 1 year [7, 12]. Therefore, early intensive (induction/intensification) chemotherapy similar to that used to treat AML should be administered, even in GS patients who did not present AML upon initial diagnosis .
The authors described two cases of isolated granulocytic sarcoma of the pancreas. The experience of these cases highlighted the difficulties of correct diagnosis and care. To conclude, pretherapeutic biopsies should be the cornerstone for the diagnosis of a pancreatic mass with atypical clinical presentation.
Written informed consent was obtained from the patient for publication of this case report and the accompanying images. For the patient who died, consent was sought from the next of kin of the patient.
The authors thank Dr. Claire Delattre and Dr. Marion Classe from the Department of Pathology, University Hospital of Lille, for their help in collecting and reviewing the histological data.
- Swerlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW: WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. 2008, Lyon, France: IARC press, fourthGoogle Scholar
- Fujieda A, Nishii K, Tamaru T, Otsuki S, Kobayashi K, Monma F, Ohishi K, Nakase K, Katayama N, Shiku H: Granulocytic sarcoma of mesentery in acute myeloid leukemia with CBFB/MYH11 fusion gene but not inv(16) chromosome: case report and review of literature. Leuk Res. 2006, 30: 1053-7. 10.1016/j.leukres.2005.11.003.View ArticlePubMedGoogle Scholar
- Byrd JC, Edenfield WJ, Shields DJ, Dawson NA: Extramedullary myeloid cell tumors in acute nonlymphocytic leukemia: a clinical review. J Clin Oncol. 1995, 13: 1800-16.PubMedGoogle Scholar
- Marcos HB, Semelka RC, Woosley JT: Abdominal granulocytic sarcomas: demonstration by MRI. Magn Reson Imaging. 1997, 15: 873-6. 10.1016/S0730-725X(97)00042-8.View ArticlePubMedGoogle Scholar
- Neiman RS, Barcos M, Berard C, Bonner H, Mann R, Rydell RE, Bennet JM: Granulocytic sarcoma: a clinicopathologic study of 61 biopsied cases. Cancer. 1981, 48: 1426-37. 10.1002/1097-0142(19810915)48:6<1426::AID-CNCR2820480626>3.0.CO;2-G.View ArticlePubMedGoogle Scholar
- King DJ, Ewen SW, Sewell HF, Dawson AA: Obstructive jaundice. An unusual presentation of granulocytic sarcoma. Cancer. 1987, 60: 114-7. 10.1002/1097-0142(19870701)60:1<114::AID-CNCR2820600121>3.0.CO;2-G.View ArticlePubMedGoogle Scholar
- Moreau P, Milpied N, Thomas O, Fiche M, Parys V, Paineau J, Dutin JP, Harousseau JL: Primary granulocytic sarcoma of the pancreas: efficacy of early treatment with intensive chemotherapy. Rev Med Interne. 1996, 17: 677-9. 10.1016/0248-8663(96)87155-4.View ArticlePubMedGoogle Scholar
- Schäfer HS, Becker H, Schmitt-Gräff A, Lübbert M: Granulocytic sarcoma of Core-binding Factor (CBF) acute myeloid leukemia mimicking pancreatic cancer. Leuk Res. 2008, 32: 1472-5. 10.1016/j.leukres.2008.02.017.View ArticlePubMedGoogle Scholar
- Ravandi-Kashani F, Estey E, Cortes J, Medeiros LJ, Giles FJ: Granulocytic sarcoma of the pancreas: a report of two cases and literature review. Clin Lab Haematol. 1999, 21: 219-24. 10.1046/j.1365-2257.1999.00205.x.View ArticlePubMedGoogle Scholar
- Servin-Abad L, Caldera H, Cardenas R, Casillas J: Granulocytic sarcoma of the pancreas. A report of one case and review of the literature. Acta Haematol. 2003, 110: 188-92. 10.1159/000074223.View ArticlePubMedGoogle Scholar
- Breccia M, D'Andrea M, Mengarelli A, Morano SG, D'Elia GM, Alimena G: Granulocytic sarcoma of the pancreas successfully treated with intensive chemotherapy and stem cell transplantation. Eur J Haematol. 2003, 70: 190-2. 10.1034/j.1600-0609.2003.00021.x.View ArticlePubMedGoogle Scholar
- Rong Y, Wang D, Lou W, Kuang T, Jin D: Granulocytic sarcoma of the pancreas: a case report and review of the literatures. BMC Gastroenterol. 2010, 10: 80-10.1186/1471-230X-10-80.PubMed CentralView ArticlePubMedGoogle Scholar
- Li XP, Liu WF, Ji SR, Wu SH, Sun JJ, Fan YZ: Isolated pancreatic granulocytic sarcoma: a case report and review of the literature. World J Gastroenterol. 2011, 17: 540-2. 10.3748/wjg.v17.i4.540.PubMed CentralView ArticlePubMedGoogle Scholar
- McKenna M, Arnold C, Catherwood MA, Humphreys MW, Cuthbert RJ, Bueso-Ramos C, McManus DT: Myeloid sarcoma of the small bowel associated with a CBFbeta/MYH11 fusion and inv(16)(p13q22): a case report. J Clin Pathol. 2009, 62: 757-9. 10.1136/jcp.2008.063669.View ArticlePubMedGoogle Scholar
- Sevinc A, Buyukberber S, Camci C, Koruk M, Savas MC, Turk HM, Sari I, Buyukberber NM: Granulocytic sarcoma of the colon and leukemic infiltration of the liver in a patient presenting with hematochezia and jaundice. Digestion. 2004, 69: 262-5. 10.1159/000079847.View ArticlePubMedGoogle Scholar
- Sisack MJ, Dunsmore K, Sidhu-Malik N: Granulocytic sarcoma in the absence of myeloid leukemia. J Am Acad Dermatol. 1997, 37: 308-11. 10.1016/S0190-9622(97)80378-7.View ArticlePubMedGoogle Scholar
- Breccia M, Mandelli F, Petti MC, D'Andrea M, Pescarmona E, Pileri SA, Carmosino I, Russo E, De Fabritiis P, Alimena G: Clinico-pathological characteristics of myeloid sarcoma at diagnosis and during follow-up: report of 12 cases from a single institution. Leuk Res. 2004, 28: 1165-9. 10.1016/j.leukres.2004.01.022.View ArticlePubMedGoogle Scholar
- Pileri SA, Ascani S, Cox MC, Campidelli C, Bacci F, Piccioli M, Piccaluga PP, Agostinelli C, Asioli S, Novero D, Bisceglia M, Ponzoni M, Gentile A, Rinaldi P, Franco V, Vincelli D, Pileri A, Gesbarra R, Falini B, Zinzani PL, Baccarani M: Myeloid sarcoma: clinico-pathologic, phenotypic and cytogenetic analysis of 92 adult patients. Leukemia. 2007, 21: 340-50. 10.1038/sj.leu.2404491.View ArticlePubMedGoogle Scholar
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.