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Case report
Primary Granulocytic sarcoma Lip in a
nonleukemic patient - A rare presentation
Srivastava kirti, Paul Sayan, Gupta Deepak,Tripathi A K*,Goel Madhu Mati**Pant
MC,Verma Jitendra. Department of Radiotherapy ,*Department of Medicine ,**
Department of Pathology. CSMMU (formerly KGMU),lucknow
Abstract- A sixty year old male presented with a swelling in the
upper lip. On cytological examination patient was diagnosed as a
case of Granulocytic Sarcoma. His bone marrow examination was
normal suggesting no systemic disease. Patient achieved complete
response by radiotherapy alone. Eighteen months following
treatment patient is completely asymptomatic.
Key Words: Granulocytic Sarcoma, Radiotherapy
Introduction Granulocytic sarcoma (GS) also called
myeloblastoma or myeloid sarcoma is a rare solid tumor composed
of immature cells. It was first described by A. Burns in 1811 ( 5 ).
GS is a rare occurrence with an estimated incidence of .7 / million
1
2. in children and 2/ million in adults this tumor occurs in about 5%
of adults and 13% of children with myeloid Leukemia . In autopsy
series GS occurs in 2-8% of patients with acute and chronic
myelogenous leukemia. Such tumors often display a greenish color
due to the enzymatic action of myeloperoxidase in the tumor cells ,
hence the term “Chloroma” was given to this lesion in 1853.More
frequently the term Extra –medullary myeloid tumor (EMMT ) has
been proposed to include all the forms of extramedullary myeloid
leukemia infiltrates.. They occur most commonly in bones,
periostium , soft tissues, Lymph Nodes & skin but can occur
virtually anywhere. It is extremely rare in Oral cavity and often
present as a lump in the gingiva, palate and extraction sockets. In
view of the rarity of its occurrence and diagnostic importance we
report here a case of primary GS presenting as a solitary lump in
the lip with out bone marrow involvement.
Case History A sixty year old male patient presented to our
OPD with a history of gradually progressive swelling in right
upper lip for one month which was not associated with bleeding or
pain and no difficulty in chewing. On examination a lumpy
swelling was noted in right upper lip with redness. ( fig:1 )The
lump was non tender and there was no bleeding from the lump it
was soft to firm in consistency. The lump was mainly present on
right side while it had crossed the midline to involve medial 2/3rd
of left side of upper lip. There were some necrotic areas on the
right side of buccal mucosa with surrounding odema with mild
tenderness without any bleeding .Cytological examination of the
swelling was done suggesting granulocytic sarcoma featuring
scattered single large cells with high N:C ratio, oval to round to
irregular concentrically placed nuclei with granular chromatic &
prominent single to multiple nucleoli. The cells have scanty to
moderate amount of basophilic cytoplasm. Some of the cells
contain cytoplasmic granule, background is of RBCs & neutrophils
and lymphocytes ( Fig 2 ). A second opinion was taken from the
Pathology department of our Institution where it was confirmed to
2
3. be granulocytic sarcoma .After discussing the case in tumor board
patient received local radiotherapy 30 Gy in 15# @ 200 cGys / #
by Co-60 by parallel and opposed lat fields. Patient achieved
complete response following completion of radiotherapy he was
then advised for chemotherapy. Due to some financial constraints
patient did not receive chemotherapy but was on regular follow up.
Eighteen months following completion of radiotherapy patient is
completely asymptomatic ( Fig 3. ). His bone marrow and General
blood picture is normal till date.
Discussion: Granulocytic sarcomas often pose a diagnostic
dilemma for the oncologists. Granulocytic sarcoma (also called
myeloblastoma or myeloid sarcoma) is a rare solid tumor
composed of immature myeloid cells. It was first described in
1811.1
Such tumors often display a greenish color due to the
enzymatic action of myeloperoxidase in the tumor cells; hence, the
term "chloroma" was given to this lesion in 1853.2,3
The WHO
classification separates these tumors into myeloid or monocytic
sarcomas. Most tumors have been observed in patients with acute
myelogenous leukemia and myeloproliferative disorders3
; however,
they have been described in patients with myelodysplasia4
and
chronic lymphocytic leukemia.5,6
Granulocytic sarcomas may also
occur in the absence of leukemia,7
as notedin our patient. The most
common sites of involvement are bone, periosteum, soft tissue,
lymph node, and skin.3
Rare occurrences in muscle,8
meninges,9
breast,10
mediastinum, and ovary11
have also been reported. The
present case demonstrates the unusual extramedullary presentation
with lip involvement. This often predates involvement of the
marrow but in our case eighteen months following treatment
patient is absolutely normal. Though Granulocytic sarcoma is
treated as acute myelogenous leukemia with systemic
chemotherapy with or without local radiotherapy , Local radiation
therapy may be necessary in selected cases, like the case we have
reported .
3
4. References:
1. Menasce LP, Banerjee SS, Beckett E,Harris M. Extra-medullary myeloid tumour (
granulocytoma ) is often misdiagonosed: A study of 26 cases.Histopathology
1999;34:391-8.
2. Yamauchi K,Yasuda M comparison in treatments of nonleukemic granulocytic
sarcoma:report of two cases and a review of 72 cases in the literature.Cancer 2002
Mar 15;94(6):1739-46.
3. Byrd JC, Edenfield JW,Shields DJ,et al: Extramedullary myeloid tumors in acute
non lymphocytic myeloid leukemia: A Clinical review.J Clin Oncol 13: 1800,
1995.
4.
5.Burns A: Observations of Surgical Anatomy, Head and Neck. Edinburgh, United
Kingdom, Thomas Royce and Co, 1811, pp 364-366
6.. King A: A case of chloroma. Monthly J Med 17:97, 1853
7. Neiman RS, Barcos M, Berard C, et al: Granulocytic sarcoma: A clinicopathologic
study of 61 biopsied cases. Cancer 48:1426-1437, 1981[CrossRef][Medline]
8. List AF, Gonzalez-Osete G, Kummet T, et al: Granulocytic sarcoma in
myelodysplastic syndromes: Clinical marker of disease acceleration. Am J Med 90:274-
276, 1991[Medline]
9. Majumdar G, Singh AK: Cord compression: A rare complication of chronic
lymphocytic leukaemia. J Clin Pathol 45:258-259, 1992[Abstract/Free Full Text]
10. Michalevicz R, Burstein A, Razon N, et al: Spinal epidural compression in chronic
lymphocytic leukemia. Cancer 64:1961-1964, 1989[Medline]
11. Buckland ME, Scolyer RA, Donellan MB, et al: Spinal chloroma presenting with
triplegia in an aleukaemic patient. Pathology 33:386-389, 2001[Medline]
12. Bassichis B, McClay J, Wiatrak B: Chloroma of the masseteric muscle. Int J Pediatr
Otorhinolaryngol 53:57-61, 2000[Medline]
13. Binder C, Tiemann M, Haase D, et al: Isolated meningeal chloroma (granulocytic
sarcoma): A case report and review of the literature. Ann Hematol 79:459-462,
2000[Medline]
14. Ngu IWY, Sinclair EC, Greenaway S, et al: Unusual presentation of granulocytic
sarcoma in the breast: A case report and review of the literature. Diagn Cytopathol 24:53-
57, 2001[Medline]
4
5. 15. Sreejith G, Gangadharan VP, Elizabeth K, et al: Primary Granulocytic sarcoma of the
ovary. Am J Clin Oncol 23:239-240, 2000[Medline
Fig 2. Microphotograph from cytological smear of Fine needle aspirate from the lip
swelling smear showing immature myeloid cells including blasts. ( May Grunwald
Giemsa X 400 )
5