Japanese Journal of Clinical Oncology 31:86-88 (2001)
© 2001 Foundation for Promotion of Cancer Research
Facial Nerve Paralysis and Paraplegia as Presenting Symptoms of Acute Myeloid Leukemia
Departments of 1Hematology–Oncology and 2Pathology, Erciyes University School of Medicine, Kayseri, Turkey
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ABSTRACT |
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Granulocytic sarcoma is an extramedullary tumor associated with acute or chronic leukemias or myeloproliferative disorders. Rarely, the tumor may be seen before the diagnosis of leukemia. Symptomatic facial nerve paralysis and spinal cord invasion by granulocytic sarcomas are also relatively uncommon. We present here a 17-year-old-female patient who had facial nerve paralysis and paraplegia due to granulocytic sarcoma as the presenting symptoms of acute myeloid leukemia.
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INTRODUCTION |
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TOPABSTRACTINTRODUCTIONCASE REPORTDISCUSSIONREFERENCES |
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Granulocytic sarcoma (GS) is a localized tumor composed of immature cells of the granulocytic series. Most cases of GS occur associated with acute or chronic leukemias or myeloproliferative disorders (1). Rarely, the tumors may be observed before the diagnosis of any hematological malignancy (2,3) and most of them are harbingers of existing or impending acute myeloid leukemia (AML). GSs may occur in almost every part of the body (1,4), but spinal cord invasion and symptomatic facial nerve paralysis are relatively uncommon (5–7). We present here a 17-year-old-female who had facial nerve paralysis and paraplegia due to granulocytic sarcoma as the presenting symptoms of AML.
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CASE REPORT |
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TOPABSTRACTINTRODUCTIONCASE REPORTDISCUSSIONREFERENCES |
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A 17-year-old female was examined by a local otorhinolaryngologist in March 1999 because of acute left facial nerve paralysis. She had received corticosteroid therapy. The patient was presented to a neurosurgeon in April 1999 because of paraplegia and continuing facial nerve paralysis. Physical examination revealed loss of sensation below the thoracal 4 (Th 4) level, flaccid paralysis of the lower extremities, absence of deep tendon reflexes and rectal sphincter tone. Laboratory investigations were as follows: Hb, 11.7 g/dl; WBC, 13.3 x 109/L; platelet count, 122 x 109/L; no knowledge about the peripheral blood smear at that time. Magnetic resonance imaging (MRI) showed an extramedullary–intradural mass at the Th 4 level (Fig. 1). The mass lesion was excised totally to obtain decompression, but there was no recovery of paraplegia in the postoperative period.
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Histopathological examination of the tumor showed various mature and immature granular cells and large mononuclear cells which had lobulated nucleus and immature structure of chromatin (Fig. 2). These cells stained positively with leukocyte common antigen (LCA), CD68 and lysozyme (Figs 3,45). The tumor was diagnosed as granulocytic sarcoma and the patient was examined by the Hematology Department. Hemoglobin was 10.5 g/dl, WBC 16.7 x 109/L, differential count, 20% myelo-monoblastic cells, 35% mature lymphocytes and 45% granulocytes and the platelet count was 74 x 109/L. A bone marrow aspiration revealed diffuse infiltration of myelo-monoblastic cells (the distribution of the non-erythroid cells was 70% myelo-monoblasts, 5% promyelocytes, 20% myelocytes, metamyelocytes and neutrophils, 4% lymphocytes and 1% eosinophils). Blasts were positive with Sudan Black and periodic acid–Schiff (PAS) staining. With these findings, the patient was diagnosed as AML-M4 according to the French–American–British (FAB) classification. Temporal MRI showed bilateral maxillary sinus involvement by the tumor and bilateral mastoiditis.
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The patient received adriamycin (25 mg/m2/day) for 3 days and cytarabine (150 mg/m2/day) for 7 days. After two courses of chemotherapy, hematological partial remission was obtained. Facial paralysis recovered, but there was no improvement in paraplegia. After the second course of chemotherapy the patient died because of sepsis associated with extensive decubitus ulcers.
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DISCUSSION |
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TOPABSTRACTINTRODUCTIONCASE REPORTDISCUSSIONREFERENCES |
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GS and its association with AML are well known entities. Most of the patients are children and young adults. Generally, survival of the cases with GS is shorter than that of the patients without GS who received the same treatment (8).
GS may come out with various signs and symptoms, which may be an indicator of blastic phase in chronic leukemia or as a leukemic transformation in myeloproliferative disorders. Some cases may be seen as isolated cases without any hematological disorder. In nearly half of these patients acute leukemia develops in a short time (3). In our patient facial nerve paralysis preceded the diagnosis of acute leukemia nearly 1 month earlier and extramedullary–intradural tumoral mass causing paraplegia preceded 1 week earlier. After the detection of GS, peripheral blood smear and bone marrow aspiration revealed the diagnosis of AML. It could not be established whether there was acute leukemia initially, because the evaluation of peripheral blood smear and bone marrow aspiration were not performed during the first two visits.
GS shows differences in histopathological appearance as well as in clinical progress. In particular, patients without antecedent hematological disease may be diagnosed as lymphoma at the first evaluation. Staining with naphthol ASD chloroacetate esterase and the antilysozyme immunoperoxidase technique should be performed in suspicious cases (1). In our case, histopathological examination of the epidural mass revealed immature giant mononuclear cells and various mature and immature granular cells staining positively with LCA, CD68 and lysozyme. Naphthol ASD chloroacetate esterase stain could not be performed because of the tissue fixation.
Central nervous system symptoms may occur due to GS in patients with AML (9). In granulocytic tumors causing spinal cord compression, the treatment of choice is to try radiotherapy and/or chemotherapy before surgery (10). Most of the tumors shrink or disappear with these approaches. There are also several reports concerning the efficacy of radiotherapy and chemotherapy in cases without acute leukemia. In addition, prolonged survival and reduced risk for development of AML were obtained in patients with isolated GS receiving chemotherapy. However, the prognosis did not change for the patients who underwent RT or surgical treatment (11). Our patient was admitted to hospital with acute paraplegia. Initially the mass was considered as a primary central nervous system tumor and it was excised totally to obtain decompression. However, paraplegia did not improve after surgical resection.
Symptomatic facial nerve involvement in AML patients is rare (6,7). It may be due to leukemic cell infiltration at any site of the facial nerve or occurrence of GS in temporal bone or auditory canal and may be accompanied by hearing loss (6). Our patient had only facial nerve paralysis and not hearing loss and MRI showed bilateral infiltration in maxillary sinuses and mastoiditis which may indicate leukemic involvement.
The treatment for facial nerve involvement is chemotherapy and/or radiotherapy (7). Facial paralysis improved clinically with chemotherapy in our patient. Control MRI could not be performed because she died while receiving chemotherapy.
In the medical literature, we could not find any report of such a patient presenting with multiple neurological findings associated with GS before the diagnosis of AML. As in this case, patients may enter hospital with various neurological signs including paraplegia and facial nerve palsy before diagnosis of AML. GS and leukemic infiltration should be considered in the differential diagnosis, particularly in pediatric patients and in young adults, and bone marrow aspiration should be done. In patients with GS and neurological symptoms, chemotherapy and/or radiotherapy should be tried prior to surgery (except in emergency conditions) because of the good response.
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FOOTNOTES |
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+ For reprints and all correspondence: Bulent Eser, Department of Hematology–Oncology, Erciyes University School of Medicine, 38039 Kayseri, Turkey. E-mail: beser@erciyes.edu.tr
Abbreviations: GS, granulocytic sarcoma; AML, acute myeloid leukemia; Th, thoracal; Hb, hemoglobin; WBC, white blood cell; MRI, magnetic resonance imaging; LCA, leukocyte common antigen; PAS, periodic acid–Schiff; FAB classification, French–American–British classification
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REFERENCES |
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Received June 20, 2000; accepted November 16, 2000.
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