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Intradural granulocytic sarcoma: a rare cause of sciatic pain
Intradural granulocytic sarcoma: a rare cause of sciatic pain Arne Mosch*, Rudy R.F. Kuiters* *, and Bart A. Kazzaz* * *
Introduction Summary
Granulocytic sarcoma or myeloblastoma is a rare tumor associated with acute leukemia and myeloproliferative disorders. It is regarded as a true neoplasma, composed of granulocytic precursors occuring in an extramedullary site’. If tinged green it is called chloroma, or myeloblastoma when unpigmented. The color depends on the concentration of myeloperoxidase2T3. Frequently these myeloblastic tumors are localized to perineural and epineural structures. Spinal cord involvement by leukemic masses is very rare’ and is usually extradural, producing pain and signs of spinal cord compression. This report describes a sciatic syndrome due to an intradural granulocytic sarcoma within the Sl ganglion . Case Report
On August 1987 a 67-year-old man showed spontaneous hematomas as initial symptom of an acute myeloid leukemia (FAB-classication M2)4. Three months after treatment with chemotherapy he developed progressive sciatic pain in his right leg. Physical examination showed a diminished right patellar tendon reflex and bilaterally absent achilles tendon reflexes. Straight leg raising
* Department Netherlands.
of Neurology,
** Neurosurgery
and ***
Granulocytic sarcoma is a solid tumor associated with acute leukemia and myeloproliferative disorders. In this report we present a patient with a progressive sciatic pain due to an intradural granulocytic sarcoma fixed on the right spinal nerve root Sl. The incidence and significance of central nervous involvement by granulocytic sarcoma are discussed. Key words: Acute myeloid leukemia, granulocytic sarcoma (chloroma), spinal intradural tumors.
on the right side was positive, together with a reverse Ladgue. Muscle power of the legs was intact. Local pressure pain was found on the vertebra Sl. No lymphadenopathy was found. Routine laboratory tests were within normal limits. An iliac crest biopsy was normal. Cerebrospinal fluid (CSF) was obtained at a normal opening pressure, contained 710 lymphocytes and monocytes/mm3, these mostly being myelocytes, and raised level of protein (0.79 mg/l) and a B-Zmicroglobulin (2.3 mg/l). Cytologic examination showed myelocytes. Lumbar myelography showed a right lateral intradural defect with compression of the right
Pathology,
Municipal Hospitals of The Hague,
Address for correspondence and reprint requests: A. Mosch, Department of Neurology, Leyweg 275, 2545 CH The Hague, The Netherlands.
The Hague,
The
Municipal Hospital of The Hague,
Accepted 66.91 Clin Neurol Neurosurg 1991. Vol. 93-4
341
Figure
I.
Myelography
an encroachement nerve root S I placement
nerve root Sl. The radiological ptcturc was thought to be consistent with a peridural tumor (fig. 1). A second bone marrow aspirate showed a megaloblastic haematopoiesis with less than 5% blasts cells and visible effects of chemotherapy. The diagnosis of a meningeal localisation of the AML was made and treatment with intrathecal Methotrexate (MTX) was started. Control CSF-analysis still contained blastcells. A
and :I medial
di+
of the Jural sack as ii
whole
31 the Icvci
rows).
(A)
(W view
showmg
of the right
medial
Right
I.C-SI
lateral
(XIview:
.Intrroposterior
thin needle cytological puncture of the intradural. tumor revealed no blasts but showed cytological characteristics of an ependymoma. A laminectomy L5-Sl was performed on the basis of a tentative diagnosis of spinal intradural tumor showing persistence of blasts in the CFS without clinical improvement of the sciatic pain. Extradural inspection of the right nerve root Sl showed an intracanalicular, grey-brownsh. subdural tumor. After opening the dura, the tumor
Figure 2. Pathological specimen showing leukemic cells surrounding nerve roots ganglia (hematoxylin 125).
342
and eosin stain x
(measuring 1.5 cm in greatest diameter) appeared to originate from the Sl dorsal root and adhered to the dura. The complete tumor was easily separated from the surrounding structures. Pathological examination revealed immature leukemic cells surrounding the nerve roots and ganglion cells consistent with granulocytic sarcoma (fig. 2). The typical green colour of a chloroma was absent. Postoperatively the patient had immediate relief of his sciatic pain. Five days after the operation leukemic cells appeared in the peripheral blood. This relapse was treated with a high dose of Ara-C and Amsacrine, which resulted in a complete bone marrow remission. Local X-ray therapy was given to the lumbar-sacral area with adjuvant radiotherapy to the skull. Before irradiation was started no blasts were seen in the CSF. Three months later a routine bone marrow biopsy showed an increase of blasts and dysplastic haematopoiesis. Once again blasts were present in the CFS. Treatment with intrathecal MTX and oral Leucovorine followed. The patient died in September 1988 having a severe thrombocytopenia and a white blood count of 180x 109/1 (with 98% myeloblasts). Autopsy findings: localisation of AML in liver, spleen, kidney, adrenal, lungs and pituitary gland. Leukostasis was found in all cerebral arteries and veins. Discussion
Granulocytic sarcoma (chloroma) is an uncommon finding, clinically presenting in 2.9% of all acute leukemic cases. It is strongly associated with acute myelogenous leukemia (AML) or the accelerated phase of chronic myelogenous leukemia (CML) 1~2,3 . The most common sites of involvement are bone, periosteum, soft tissue, lymphnodes and skin’. Spinal cord involvement by chloroma is very rare (0.8%), with a predilection for younger patient$‘. The thoracic area is most commonly affected7. The most common presentation is an extradural leukemic infiltration3,‘,‘. Presenting symptoms are back pain, radicular pain, symptoms of cord compression or rarely a conus medullaris syndrome. Intradural granulocytic sarcoma originating from nerve roots is extremely rare Il~.ll.l?.
Both acute lymphocytic and myelocytic leukemias can involve the walls of arachnoidal veins, the dura and the arachnoidal connective tissue, this permitting direct shedding into the CSF. Later there may be a disruption of pia-glial membrane in the Virchow-Robin space’3,‘4. This latter feature is believed to form granulocytic sarcomas in the CNS. Previous explanations of intra- and extradural sarcomas have suggested perivascular spread of leukemic cells via the vertebral venous plexus and radicular veins, or trapping of leukemic cells in the CFS by arachnoid granulations of the nerve root sleeve”. The total prevalence of leukemic infiltrations of the meninges rose with the increasing lenght of survival after the introduction of effective chemotherapy. Autopsy studies of AML patients established that 25% to 50% had leukemic dural or meningeal infiltration, while only in 34% of autopsy studies a granulocytic sarcoma was reported’5,‘6.‘7. Granulocytic sarcomas most often present in patients already known with AML, active or in remission. When the patient is in complete hematological remission the occurrence of granulocytic sarcoma appears to be often the initial manifestation of a blast transformation, as was the case in our patient. Sometimes a granulocytic sarcoma may be the presenting feature of the disease, or it may even preceed the development of acute leukemia seen in blood and marrow analysis several months to years~~9~1s~‘9~20. The survival of patients with established AML and granulocytic sarcoma is almost similar to that of patients known with AML without tumor formation, even when the patient is in complete remission’R.“. The prognostic significance of the occurrence of granulocytic sarcoma without discernible hematologic abnormality is considerable, because it strongly suggests the development of AML”. Patients with an isolated granulocytic sarcoma (showing normal peripheral blood, CSF and bone marrow examination), who only received local treatment, were known to develop leukemia in later stages. Generally these patients are now being treated with chemotherapy as if they had AML2?. Some of these treated patients never develop AML, others die within a few months from systemic AML. Also for patients with myeloproliferative dis343
orders (CML) granulocytic sarcoma appears to have great prognostic significance. Tumor formation was either the initial manifestation of blast transformation, or a warning of AML appearing some months later (mean 6-10.5 months)3B21.
PIERCE M. Neurological compiications in acute leukemia in children. Pediatr Clin North Am 1962; 9:42S-42. WIEHYDE PH, SERPICK AA, MULLAN s. Spinal epidural leukemia. Am J Med 1970 35:361-64. PETIJRSSON SR, BOGGS DR. SpiBRl cord jnVO~VemeUtiU leukemia: a review of the literature and a case of Phi + acute myeloid leukemia presenting with a conus medullaris syndrome. Cancer 1981; 47:346-50.
SPIEGELMANNR,RAMZ,FINDLERG,KNVLLERN,SHAKED
It seems that granulocytic sarcoma is a heterogenous lesion in terms of clinical behavior, which may develop at any time during the course of myelogenous leukemia. The appearance of granulocytic sarcoma can suggest a highly aggressive and lethal form of AML or a possible curable forrr?. Patients with meninge~ leukemia as an initial presentation still appear to have less chance of entering complete remission16. The majority of patients with spinal granulocytic sarcoma underwent incisional biopsy or surgical resection of the localized tumor, followed by intrathecal chemotherapy,with or without systemic chemotherapy and local radiation therapy. The effect of spinal cord involvement of prognosis remains unclear.
I,SAHAR A. Spinal cord involvement as the presenting symptom of acute monocytic leukemia. Surg Neurol 1988; 29:145-48. EEI.K~MAE,JOHNSONDW,LATCHAW~. M~tipiespinal granuiocytic sarcomas simulating neurofibromatosis. AJNR 1989; lO:S42-544.
GLASS
JP, VAN TASSEL
WILLIAMS WJ et al. Hematology. 4’h ed. New York: McGraw-Hill, 199fhlS3. KRAUSE JR. Granulocytic sarcoma preceding acute leukemia. Cancer 197% 44:1017-21. MUSS HB, MOLOMNEY WC. Chloroma and Other Myeioblastic Tumors. Blood 1973; 42721-27. BENNETJM,CATOVSKYD,DANlELMT,FLANDRlNG,GALDAG, GRALNICK HR, SUTAN c. Proposals for the Classification of the Acute Leukaemias. Br J Haematol 1976; 33:4Sl-58. TON
~I~N~,BARCOSM,BE~A~C,BON~RH,~R, RYDELL RE, BENNET JM. Gramdocytic sarcoma: a chnicophatologic study of 61 biopsied cases. Cancer 1981; 48~1426-37.
344
E,PANTOLOGY
D. In-
P, KEATING
MJ,
CORK A, TRuJlLLO J,
Central nervous system complications of a newly recognized subtype of leukemia: AML with a pericentric inversion of chromosome 16. Neurology 1987; 37:639&t. PRICE RA, JOHNSON ww. The central nervous leukemia in childhood leukemia: the arachnoid. Cancer 1973; 31:520-33. HOLMES
R.
LIUP1,lSHIMARUT,MCGREGORDH,OKADAH,STREERA.
Autopsy study of granuiocytic sarcoma (cbloroma) in patients with myelogenous leukemia, Hirosbima-Nagasaki 1949-69. Cancer 1972; 31:948-55. RJ,FERREIRA
GOLDBERG
References
WARNER
tradural granulocytic sarcoma presenting as a lumbar radiculopathy. Case report. J Neurosurg 1990; 72,4:663667. MIRVIS s, STEWART M, RAO KCVG. Mye~ograp~ic demonstration of noduiar radiculopathy in acute myelogenous leukemia. AJNR 1984; 5:641-643.
MEYER
We thank R.E.M. Hekster, M.D., department of Neuroradiology for giving permission to publish the X-rays photographs and Linda v. Oosterhout for preparing the manuscript.
L,
BERNSTEIN M,RESCH
J, HOLLAND
PPC,ClJTTNERJ,GREENBERG
ML,
JF. C‘ZlltDit B‘XVOUS SyStelUin-
volvement at presentation in acute granulocytic leukemia. Am J Med 1980; 68:691-94. woLk aw, MASSE SR, CONKLIN R, FREIREICH EL The incidence of central nervous system leukemia adults with acute leukemia. Cancer 1971; 33863-69. BALLARD
JO, TOWFIGHI
I, BRENNAN
RW,
SALEEM
S, EYS-
Neurological complications of acute myelomonobiastic leukemia of four years’ duration. Neurology 1978; 28~174-8. COMINGSDE,FAYENAW,CARTERP. Myeloblastomapreceding blood and marrow evidence of acute leukemia. Cancer 1965; 18253-58. MASON TE, DEMARREE RS, ~LARG~LM cl. Granulocytic sarcoma (chloroma), two years preceding myelogenous leukemia. Cancer 1973; 31:423-29. MEIS JM, BUTLER JJ, OSBORNE BM, MANNING JT. Granulocytic sarcoma in Nonleukemic Patients. Cancer 58; 19862697-709. ESHGHABADI M, MAJID SHOJANIA A, CARR I. Isoiated granulocytic sarcoma: report of a case and review of the literature. J Clin Oncol 1986; 4:912-17. TER E.
Introduction Summary
Granulocytic sarcoma or myeloblastoma is a rare tumor associated with acute leukemia and myeloproliferative disorders. It is regarded as a true neoplasma, composed of granulocytic precursors occuring in an extramedullary site’. If tinged green it is called chloroma, or myeloblastoma when unpigmented. The color depends on the concentration of myeloperoxidase2T3. Frequently these myeloblastic tumors are localized to perineural and epineural structures. Spinal cord involvement by leukemic masses is very rare’ and is usually extradural, producing pain and signs of spinal cord compression. This report describes a sciatic syndrome due to an intradural granulocytic sarcoma within the Sl ganglion . Case Report
On August 1987 a 67-year-old man showed spontaneous hematomas as initial symptom of an acute myeloid leukemia (FAB-classication M2)4. Three months after treatment with chemotherapy he developed progressive sciatic pain in his right leg. Physical examination showed a diminished right patellar tendon reflex and bilaterally absent achilles tendon reflexes. Straight leg raising
* Department Netherlands.
of Neurology,
** Neurosurgery
and ***
Granulocytic sarcoma is a solid tumor associated with acute leukemia and myeloproliferative disorders. In this report we present a patient with a progressive sciatic pain due to an intradural granulocytic sarcoma fixed on the right spinal nerve root Sl. The incidence and significance of central nervous involvement by granulocytic sarcoma are discussed. Key words: Acute myeloid leukemia, granulocytic sarcoma (chloroma), spinal intradural tumors.
on the right side was positive, together with a reverse Ladgue. Muscle power of the legs was intact. Local pressure pain was found on the vertebra Sl. No lymphadenopathy was found. Routine laboratory tests were within normal limits. An iliac crest biopsy was normal. Cerebrospinal fluid (CSF) was obtained at a normal opening pressure, contained 710 lymphocytes and monocytes/mm3, these mostly being myelocytes, and raised level of protein (0.79 mg/l) and a B-Zmicroglobulin (2.3 mg/l). Cytologic examination showed myelocytes. Lumbar myelography showed a right lateral intradural defect with compression of the right
Pathology,
Municipal Hospitals of The Hague,
Address for correspondence and reprint requests: A. Mosch, Department of Neurology, Leyweg 275, 2545 CH The Hague, The Netherlands.
The Hague,
The
Municipal Hospital of The Hague,
Accepted 66.91 Clin Neurol Neurosurg 1991. Vol. 93-4
341
Figure
I.
Myelography
an encroachement nerve root S I placement
nerve root Sl. The radiological ptcturc was thought to be consistent with a peridural tumor (fig. 1). A second bone marrow aspirate showed a megaloblastic haematopoiesis with less than 5% blasts cells and visible effects of chemotherapy. The diagnosis of a meningeal localisation of the AML was made and treatment with intrathecal Methotrexate (MTX) was started. Control CSF-analysis still contained blastcells. A
and :I medial
di+
of the Jural sack as ii
whole
31 the Icvci
rows).
(A)
(W view
showmg
of the right
medial
Right
I.C-SI
lateral
(XIview:
.Intrroposterior
thin needle cytological puncture of the intradural. tumor revealed no blasts but showed cytological characteristics of an ependymoma. A laminectomy L5-Sl was performed on the basis of a tentative diagnosis of spinal intradural tumor showing persistence of blasts in the CFS without clinical improvement of the sciatic pain. Extradural inspection of the right nerve root Sl showed an intracanalicular, grey-brownsh. subdural tumor. After opening the dura, the tumor
Figure 2. Pathological specimen showing leukemic cells surrounding nerve roots ganglia (hematoxylin 125).
342
and eosin stain x
(measuring 1.5 cm in greatest diameter) appeared to originate from the Sl dorsal root and adhered to the dura. The complete tumor was easily separated from the surrounding structures. Pathological examination revealed immature leukemic cells surrounding the nerve roots and ganglion cells consistent with granulocytic sarcoma (fig. 2). The typical green colour of a chloroma was absent. Postoperatively the patient had immediate relief of his sciatic pain. Five days after the operation leukemic cells appeared in the peripheral blood. This relapse was treated with a high dose of Ara-C and Amsacrine, which resulted in a complete bone marrow remission. Local X-ray therapy was given to the lumbar-sacral area with adjuvant radiotherapy to the skull. Before irradiation was started no blasts were seen in the CSF. Three months later a routine bone marrow biopsy showed an increase of blasts and dysplastic haematopoiesis. Once again blasts were present in the CFS. Treatment with intrathecal MTX and oral Leucovorine followed. The patient died in September 1988 having a severe thrombocytopenia and a white blood count of 180x 109/1 (with 98% myeloblasts). Autopsy findings: localisation of AML in liver, spleen, kidney, adrenal, lungs and pituitary gland. Leukostasis was found in all cerebral arteries and veins. Discussion
Granulocytic sarcoma (chloroma) is an uncommon finding, clinically presenting in 2.9% of all acute leukemic cases. It is strongly associated with acute myelogenous leukemia (AML) or the accelerated phase of chronic myelogenous leukemia (CML) 1~2,3 . The most common sites of involvement are bone, periosteum, soft tissue, lymphnodes and skin’. Spinal cord involvement by chloroma is very rare (0.8%), with a predilection for younger patient$‘. The thoracic area is most commonly affected7. The most common presentation is an extradural leukemic infiltration3,‘,‘. Presenting symptoms are back pain, radicular pain, symptoms of cord compression or rarely a conus medullaris syndrome. Intradural granulocytic sarcoma originating from nerve roots is extremely rare Il~.ll.l?.
Both acute lymphocytic and myelocytic leukemias can involve the walls of arachnoidal veins, the dura and the arachnoidal connective tissue, this permitting direct shedding into the CSF. Later there may be a disruption of pia-glial membrane in the Virchow-Robin space’3,‘4. This latter feature is believed to form granulocytic sarcomas in the CNS. Previous explanations of intra- and extradural sarcomas have suggested perivascular spread of leukemic cells via the vertebral venous plexus and radicular veins, or trapping of leukemic cells in the CFS by arachnoid granulations of the nerve root sleeve”. The total prevalence of leukemic infiltrations of the meninges rose with the increasing lenght of survival after the introduction of effective chemotherapy. Autopsy studies of AML patients established that 25% to 50% had leukemic dural or meningeal infiltration, while only in 34% of autopsy studies a granulocytic sarcoma was reported’5,‘6.‘7. Granulocytic sarcomas most often present in patients already known with AML, active or in remission. When the patient is in complete hematological remission the occurrence of granulocytic sarcoma appears to be often the initial manifestation of a blast transformation, as was the case in our patient. Sometimes a granulocytic sarcoma may be the presenting feature of the disease, or it may even preceed the development of acute leukemia seen in blood and marrow analysis several months to years~~9~1s~‘9~20. The survival of patients with established AML and granulocytic sarcoma is almost similar to that of patients known with AML without tumor formation, even when the patient is in complete remission’R.“. The prognostic significance of the occurrence of granulocytic sarcoma without discernible hematologic abnormality is considerable, because it strongly suggests the development of AML”. Patients with an isolated granulocytic sarcoma (showing normal peripheral blood, CSF and bone marrow examination), who only received local treatment, were known to develop leukemia in later stages. Generally these patients are now being treated with chemotherapy as if they had AML2?. Some of these treated patients never develop AML, others die within a few months from systemic AML. Also for patients with myeloproliferative dis343
orders (CML) granulocytic sarcoma appears to have great prognostic significance. Tumor formation was either the initial manifestation of blast transformation, or a warning of AML appearing some months later (mean 6-10.5 months)3B21.
PIERCE M. Neurological compiications in acute leukemia in children. Pediatr Clin North Am 1962; 9:42S-42. WIEHYDE PH, SERPICK AA, MULLAN s. Spinal epidural leukemia. Am J Med 1970 35:361-64. PETIJRSSON SR, BOGGS DR. SpiBRl cord jnVO~VemeUtiU leukemia: a review of the literature and a case of Phi + acute myeloid leukemia presenting with a conus medullaris syndrome. Cancer 1981; 47:346-50.
SPIEGELMANNR,RAMZ,FINDLERG,KNVLLERN,SHAKED
It seems that granulocytic sarcoma is a heterogenous lesion in terms of clinical behavior, which may develop at any time during the course of myelogenous leukemia. The appearance of granulocytic sarcoma can suggest a highly aggressive and lethal form of AML or a possible curable forrr?. Patients with meninge~ leukemia as an initial presentation still appear to have less chance of entering complete remission16. The majority of patients with spinal granulocytic sarcoma underwent incisional biopsy or surgical resection of the localized tumor, followed by intrathecal chemotherapy,with or without systemic chemotherapy and local radiation therapy. The effect of spinal cord involvement of prognosis remains unclear.
I,SAHAR A. Spinal cord involvement as the presenting symptom of acute monocytic leukemia. Surg Neurol 1988; 29:145-48. EEI.K~MAE,JOHNSONDW,LATCHAW~. M~tipiespinal granuiocytic sarcomas simulating neurofibromatosis. AJNR 1989; lO:S42-544.
GLASS
JP, VAN TASSEL
WILLIAMS WJ et al. Hematology. 4’h ed. New York: McGraw-Hill, 199fhlS3. KRAUSE JR. Granulocytic sarcoma preceding acute leukemia. Cancer 197% 44:1017-21. MUSS HB, MOLOMNEY WC. Chloroma and Other Myeioblastic Tumors. Blood 1973; 42721-27. BENNETJM,CATOVSKYD,DANlELMT,FLANDRlNG,GALDAG, GRALNICK HR, SUTAN c. Proposals for the Classification of the Acute Leukaemias. Br J Haematol 1976; 33:4Sl-58. TON
~I~N~,BARCOSM,BE~A~C,BON~RH,~R, RYDELL RE, BENNET JM. Gramdocytic sarcoma: a chnicophatologic study of 61 biopsied cases. Cancer 1981; 48~1426-37.
344
E,PANTOLOGY
D. In-
P, KEATING
MJ,
CORK A, TRuJlLLO J,
Central nervous system complications of a newly recognized subtype of leukemia: AML with a pericentric inversion of chromosome 16. Neurology 1987; 37:639&t. PRICE RA, JOHNSON ww. The central nervous leukemia in childhood leukemia: the arachnoid. Cancer 1973; 31:520-33. HOLMES
R.
LIUP1,lSHIMARUT,MCGREGORDH,OKADAH,STREERA.
Autopsy study of granuiocytic sarcoma (cbloroma) in patients with myelogenous leukemia, Hirosbima-Nagasaki 1949-69. Cancer 1972; 31:948-55. RJ,FERREIRA
GOLDBERG
References
WARNER
tradural granulocytic sarcoma presenting as a lumbar radiculopathy. Case report. J Neurosurg 1990; 72,4:663667. MIRVIS s, STEWART M, RAO KCVG. Mye~ograp~ic demonstration of noduiar radiculopathy in acute myelogenous leukemia. AJNR 1984; 5:641-643.
MEYER
We thank R.E.M. Hekster, M.D., department of Neuroradiology for giving permission to publish the X-rays photographs and Linda v. Oosterhout for preparing the manuscript.
L,
BERNSTEIN M,RESCH
J, HOLLAND
PPC,ClJTTNERJ,GREENBERG
ML,
JF. C‘ZlltDit B‘XVOUS SyStelUin-
volvement at presentation in acute granulocytic leukemia. Am J Med 1980; 68:691-94. woLk aw, MASSE SR, CONKLIN R, FREIREICH EL The incidence of central nervous system leukemia adults with acute leukemia. Cancer 1971; 33863-69. BALLARD
JO, TOWFIGHI
I, BRENNAN
RW,
SALEEM
S, EYS-
Neurological complications of acute myelomonobiastic leukemia of four years’ duration. Neurology 1978; 28~174-8. COMINGSDE,FAYENAW,CARTERP. Myeloblastomapreceding blood and marrow evidence of acute leukemia. Cancer 1965; 18253-58. MASON TE, DEMARREE RS, ~LARG~LM cl. Granulocytic sarcoma (chloroma), two years preceding myelogenous leukemia. Cancer 1973; 31:423-29. MEIS JM, BUTLER JJ, OSBORNE BM, MANNING JT. Granulocytic sarcoma in Nonleukemic Patients. Cancer 58; 19862697-709. ESHGHABADI M, MAJID SHOJANIA A, CARR I. Isoiated granulocytic sarcoma: report of a case and review of the literature. J Clin Oncol 1986; 4:912-17. TER E.