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.
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.
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