Granulocytic sarcoma of gingiva: an unusual case with aleukemic presentation

BACKGROUND: Granulocytic sarcoma (GS) is an uncommon extramedullary tumor composed of dense aggregates of immature myeloid precursor cells, which is usually associated with acute or chronic myeloid leukemia. The tumor may also be a predecessor to acute myeloid leukemia (AML). It may be found in any location throughout the body; however, intraoral occurrence is extremely rare. This report describes a case of gingival granulocytic sarcoma that developed prior to AML in a 12-year-old female. METHODS AND RESULTS: The patient, who had a 3-month history of gingival enlargement, was diagnosed as having granulocytic sarcoma based on clinical, radiological, and histological findings. Although the tumor regressed significantly after two induction chemotherapy courses, the patient subsequently died due to pneumococcal sepsis and pleurisy. CONCLUSION: This case report affirms the importance of granulocytic sarcoma in the differential diagnosis of gingival enlargements, since the tumor may occur before bone marrow involvement by leukemic cells.     

Acute myelogenous leukemia: advances and limitations of treatment

Acute myelogenous leukemia (AML) describes a group of related hematologic malignancies that are being approached therapeutically from several perspectives. Conventional chemotherapeutic agents, such as anthracyclines and cytosine arabinoside (Ara-C), are useful in treating AML but now appear to have reached their maximum potential. Newer therapeutic approaches to AML have recently focused on immune-based therapy through monoclonal antibodies that target and destroy malignant cells via specific cell receptors. One such agent is gemtuzumab (CMA-676), an agent that targets the CD33 antigen on malignant myeloid cells. Initial studies have shown significant anticancer activity. We will discuss traditional and newer therapeutic approaches to AML and review the role of monoclonal antibody based therapies for patients with AML. A case of a 30-year-old man with refractory AML who was treated with gemtuzumab will be mentioned, highlighting potential applications and possible limitations to this novel therapy. Despite the effective reduction in the number of malignant cells in bone marrow, gemtuzumab ineffectively treated extramedullary leukemic gingival infiltrate. Regardless of limitations, monoclonal-based therapy offers an exciting and potentially safer adjunctive therapy for patients with AML.     

Rare labial ulcer related to the use of all‐trans retinoic acid in a patient with acute promyelocytic leukemia

Acute promyelocytic leukemia (APL) is a subtype of acute myeloid leukemia (AML). All‐trans retinoic acid (ATRA) is the first‐choice therapy for the treatment of this disease, but has been associated with side effects, the most serious of which is retinoic acid syndrome (RAS). RAS is characterized by unexplained fever, dyspnea, pulmonary infiltrate, leukocytosis and nephropathy. Genital ulcers have been described in some cases, but only two cases of oral ulcers related to this syndrome have been described in the literature. This paper describes the third case of oral ulceration related to ATRA in a 32‐year‐old white man with diagnosis of APL. Clinicians should know the side effects of ATRA and identify oral ulcers resulting from this therapy. The prompt identification of these ulcers enables the institution of appropriate treatment and can therefore contribute to continuation of the patient's cancer treatment.     

Analysis of oral manifestations of leukemia: a retrospective study

OBJECTIVE: To evaluate in detail the oral complications of leukemia at initial presentation. Associations between oral manifestations and laboratory data were also investigated.
STUDY DESIGN: A retrospective study of 230 cases was conducted to investigate the nature and incidence of oral manifestations at initial presentation. The following data were determined for each patient: age, sex, haemogram at diagnosis (which included haemoglobin, red blood cell counts, differential white blood cell counts, platelet counts), classification of the type of leukemia's systemic symptoms and oral manifestations at the initial physical examination including the dental consultation.
RESULTS: It was revealed that the type of leukemia diagnosed, except with CML, is related to the age prediliction of patients. The incidence of leukemia is higher in males than in females. The most common manifestations of leukemia are lymphadenopathy (71.4% in ALL; 45% in AML), laryngeal pain (52.7% in ALL; 37.3% in AML), gingival bleeding (43.2% in AML; 28.6% in ALL), oral ulceration, and gingival enlargement. Fever (92.2%) was the most common symptom in patients with all types of leukemia. Platelet counts from 25000 mm^-3 to 60000 mm^-3 are at sufficiently low levels to result in spontaneous bleeding. Most of the patients had WBC counts of greater than 10000 mm^-3.Only 12.6% of patients had normal WBC counts.
CONCLUSIONS: It was concluded that the age predilic-tion and prevalence of oral manifestations are closely related to the type of leukemia.     

A report of oral extramedullary acute myeloid leukaemia (AML) in an 8-year-old girl with newly diagnosed AML-M4Eo.

Acute myeloid leukaemia (AML), characterized by proliferation of immature neoplastic myeloid cells, is uncommon in childhood. We present a case of an 8-year-old girl with AML-M4Eo who had an extramedullary leukaemic tumour in the oral cavity.     

Leukemic gingival infiltrate as an indicator of chemotherapeutic failure following monoclonal antibody therapy: a case report

Treatment options are limited for patients with relapsed acute myelogenous leukemia (AML), particularly when the disease is refractory to standard cytotoxic chemotherapy. Targeted drug therapy offers the advantage of delivering higher doses of non-cross-resistant chemotherapy with potentially less systemic toxicity. Gemtuzumab ozogamicin (Mylotarg, Wyeth-Ayerst Laboratories) is an immunoconjugate that consists of humanized anti-CD 33 antibody linked to the potent anti-tumor antibiotic calicheamicin and has been an effective therapy for some patients with relapsed AML. However, the overall utility of gemtuzumab ozogamicin is not well defined. For instance, it is not known how well this antibody will target extramedullar/ disease. This article reports gemtuzumab treatment of refractory AML in a 32–year-old man. At the time of recurrence, his bone marrow was hypoplastic and without leukemia, but the condition progressed resulting in marked leukemic infiltration of the oral mucosa. This case history raises the possibility that leukemic sanctuary sites may exist, and that monoclonal antibody therapy may have sub-optimal activity in non-medullary sites of disease.     

急性髓系白血病患者化疗前口腔治疗预防化疗性口腔黏膜炎的初步研究

目的探讨急性髓系白血病患者化疗前给予口腔治疗对化疗期间口腔黏膜炎发生率及炎症程度的影响,为临床造血系统恶性肿瘤患者化疗前口腔治疗效果提供初步依据。 方法74例患有急性髓系白血病的患者根据患者是否愿意化疗前接受口腔治疗,分为口腔治疗+化疗组和化疗组,口腔治疗+化疗组在化疗前给予全面的口腔检查以及对存在问题进行处理,化疗组则不给予任何口腔治疗。评价和记录口腔黏膜炎的发生率及程度。采用卡方检验及Mann-Whitney U检验,比较两组间差异。 结果口腔治疗+化疗组的口腔黏膜炎发生率要小于化疗组(χ²=5.659,P=0.017),炎症程度也低于化疗组(U=0,P=0)。 结论化疗前口腔治疗有利于减低急性髓系细胞患者化疗期间口腔黏膜炎的发生率,减轻口腔黏膜炎的炎症程度,具有重要的临床意义。     

Granulocytic sarcoma (chloroma) of the oral cavity: a case with aleukemic presentation

A case of granulocytic sarcoma, or chloroma, of the palatal mucosa, which developed 15 months before the onset of acute myelogenous leukemia (AML), is reported. The diagnosis was suspected on the basis of the light microscopic findings and confirmed by histochemical studies. Granulocytic sarcomas are rare, may be observed in a variety of body locations, and are considered specific lesions of AML or of the onset of blast crisis in chronic myelogenous leukemia. Primary granulocytic sarcomas of the oral cavity without systemic manifestations of AML are extremely rare. Clinical diagnosis of these lesions in patients with normal peripheral blood and bone marrow may be very difficult.     

Mucocutaneous granulocytic sarcomas of the head and neck

Granulocytic sarcomas are malignant solid tumors composed of poorly differentiated myeloid cells that occur in association with myelocytic leukemia. The clinicopathologic characteristics of granulocytic sarcomas that presented in the oral mucosa and in the facial, scalp and neck skin of 8 patients in the acute phase of myelocytic leukemia are described and discussed. Although most of the tumors were demonstrably sensitive to cytosine arabinoside, alone or in combination with other antineoplastic drugs, the prognostic portent of these lesions was extremely poor.     

Mucocutaneous granulocytic sarcomas of the head and neck

Granulocytic sarcomas arc malignant solid tumors composed of poorly differentiated myeloid cells that occur in association with myelocytic leukemia. The clinicopathologic characteristics of granulocytic sarcomas that presented in the oral mucosa and in the facial, scalp and neck skin of 8 patients in the acute phase of myelocytic leukemia are described and discussed. Although most of the tumors were demonstrably sensitive to cytosine arabinoside, alone or in combination with other antineoplastic drugs, the prognostic portent of these lesions was extremely poor.     

Acute myelofibrosis and multiple chloromas of the mandible and skin

Acute myelofibrosis is a rare clinical entity characterized by severe marrow fibrosis and peripheral blood pancytopenia. It may be very closely related to and has been considered synonymous with megakaryocytic leukemia. Chloroma is also a rare entity composed of a localized collection of immature myeloid cells and is associated with a known or covert leukemia. A patient presenting with a rare combination of acute myelofibrosis, megakaryocytic leukemia and multiple chloromas of the mandible and skin, are described. Although presenting a diagnostic challenge, their combination in one patient aids in the understanding of the myeloproliferative process. To our knowledge, this is the 3rd case of chloroma involving the oral osseous structures.     

Mandibular myelosarcoma (chloroma): primary oral manifestation of promyelocytic leukemia

The case of a 35-year-old female patient with primary mandibular manifestation of myelosarcoma (chloroma) is reported. The clinical course and diagnostic and therapeutic measures are described. Myelosarcomas are rare and are considered specific lesions of an acute myeloid leukemia. In addition to systemic leukemia therapy, myelosarcomas frequently have to be treated surgically or by irradiation because of their specific tumor localizations.     

Oral complaints in patients with acute myeloid leukemia treated with allogeneic hematopoietic stem cell transplantation

Background Acute myeloid leukemia belongs to proliferative diseases of the hematopoietic system. It is currently the leading indication for allogeneic hematopoietic stem cell transplantation. This study was designed to determine the most common subjective oral mucosa complaints in patients with acute myeloid leukemia after allogeneic hematopoietic cell transplantation, in relation to the type of conditioning used.Material and Methods Eighty patients diagnosed with acute myeloid leukemia were assigned to two groups depending on the intensity of the conditioning regimen before transplantation: myeloablative and reduced-intensity chemotherapy. The oral symptoms were evaluated based on an authorial questionnaire designed for this analysis. The following oral mucosa subjective complaints were included: pain, paraesthesia, burning mouth sensation, taste disorders, excessive salivation, halitosis, and dryness of the oral mucosa.Results The most commonly reported subjective oral complaint in the examined patients was xerostomia, which was found in 92% of patients during the second visit, followed by spontaneous pain in the mouth (55%), burning (36%), and dysgeusia (20%). It occurred significantly more frequently in patients who underwent myeloablative conditioning. Moreover, it was observed that the frequency of complaints increased considerably after the transplantation, reaching a peak intensity during the second week following the procedure.Conclusions Oral complaints significantly decrease the patients'' quality of life during the transplantation and may lead to premature termination of the treatment. As the number of transplantations in patients with acute myeloid leukemia increases, further investigations of oral complaints and symptoms induced by the disease itself and by the therapeutic approaches are required. Key words:Acute myeloid leukemia, oral pathology, xerostomia, myeloablative conditioning, reduced-intensity conditioning.     

Oral manifestations of acute myelomonocytic leukemia: a case report and review of the classification of leukemias

BACKGROUND: Oral signs and symptoms may indicate a serious underlying systemic disease. The most frequently observed oral findings of leukemia are mucosal bleeding and ulceration, petechiae, and gingival hyperplasia. This case report describes a 53-year-old male who presented with gingival enlargement and bleeding, fatigue, and recent weight loss as initial manifestations of acute myelomonocytic leukemia. METHODS: A gingival biopsy was performed, revealing the presence of a hypercellular infiltrate of atypical myeloid and monocytic cells. Further work-up consisted of a complete blood count, bone marrow biopsy, and immunohistochemical and histochemical analysis of biopsy material and flow cytometry of peripheral blood. RESULTS: Flow cytometry results confirmed that the infiltrate was of a myelomonocytic origin, and a diagnosis of acute myelomonocytic leukemia was rendered. The patient responded well to a chemotherapeutic induction regimen of cytosine arabinoside and idarubicin hydrochloride, with regression of gingival enlargement and remission of disease. The patient continued with consolidation chemotherapy and an autologous bone marrow transplant, but eventually died 22 months after initial diagnosis. CONCLUSIONS: Oral health care professionals, especially periodontists, must recognize that gingival enlargement may represent an initial manifestation of an underlying systemic disease. Acute myelogenous leukemia is a hematological disorder with a predilection for gingival involvement.     

Gingival infiltration in acute monoblastic leukaemia

Acute myeloid leukaemias (AML) are aggressive haematopoietic neoplasms that, if untreated, can lead to death within days. Up to 40% of presenting patients show evidence of extramedullary involvement (EMI) at diagnosis. EMI is reportedly most prevalent in myelo-monoblastic and monoblastic subtypes of AML (M4 and M5 according to FAB classification) and can present as leukaemic infiltrates in many sites including gingival enlargement and mucosal and skin nodules. We report a case of a patient who presented with gingival enlargement secondary to leukaemic infiltration. This case shows the importance of awareness of leukaemic infiltration as a cause for gingival enlargement.     

Gingival hyperplasia complicating acute myelomonocytic leukemia

Many systemic illnesses manifest clinical signs in the oral cavity. A remarkable case of gingival hyperplasia heralding the presence of acute myelomonocytic leukemia (AML FAB-M4) is described. The oral manifestations of acute leukemia are reviewed.     

Gingival fine needle aspiration cytology in acute leukemia

BACKGROUND: Oral findings in acute leukemia (AL) are common and could be the presenting feature of the disease, namely, gingival enlargement, ulceration, bleeding, and infection. Gingival enlargement in AL is either due to leukemic infiltration, or due to reactive hyperplasia. To differentiate between them a biopsy is required, but being highly contraindicated, biopsy has been substituted in this study by fine needle aspiration cytology (FNAC). METHOD: Gingival FNAC was performed on different sites in the upper and lower gingiva. Each site represents an interdental papilla, which was selected according to the clinical presentation, i.e., being enlarged or not. Seventy-two adult AL patients received a cytological and clinical examination in this study, and the cases were classified and categorized according to the French-American-British (FAB) criteria. RESULTS: Twenty-one cases were diagnosed as being infiltrated, 16 with gingival enlargement, 4 with no evidence of enlargement. In one case the infiltration affected the alveolar mucosa of an edentulous patient. In six cases the ginigva was enlarged without being infiltrated (reactive hyperplasia). Leukemic gingival enlargement was seen mostly in patients with acute myeloid leukemia, particularly M4 and M5 subtypes; however, two patients with acute mixed lineage leukemia (AMLL) were both affected with leukemic infiltration. CONCLUSION: FNAC was shown to be a simple, non-traumatic and useful diagnostic procedure for screening leukemic infiltration in gingival tissues in AL patients.     

Myeloid sarcoma occurring concurrently with drug-induced gingival enlargement

BACKGROUND: Myeloid sarcoma is an extramedullary malignancy of myeloblasts. An unusual case of myeloid sarcoma presenting in the gingiva and affected by drug-induced gingival enlargement is presented. METHODS: A 63-year-old male taking amlodipine for his hypertension presented with a 3-week gingival enlargement. Although the obvious clinical impression was that of drug-induced gingival enlargement, an incisional biopsy was performed to corroborate chemical enlargement while ruling out diseases such as lymphoma and leukemia. RESULTS: Microscopic examination of the thickened gingiva revealed surface stratified squamous epithelium having needle-like rete pegs characteristic of drug-induced gingival enlargement. Beneath the surface epithelium, the fibrous tissue was virtually replaced by a dense infiltrate of malignant cells. Immunohistochemical studies were performed with CD117 and myeloperoxidase identifying the malignant cell population as myeloblasts, leading to a diagnosis of myeloid sarcoma. CONCLUSION: Myeloid sarcoma and hematopoietic malignancies must be included in a differential diagnosis of gingival enlargement until the definitive diagnosis is reached by histologic/laboratory examination.     

Sporadic oral angiomyolipoma. Case report

Angiomyolipoma (AML) is a rare, benign tumour composed of a variable proportion of lipocytes, smooth muscle and thick-walled blood vessels. AML is part of a family of tumours arising from perivascular epithelioid cells (PEComas), and many cases are associated with tuberous sclerosis, with the kidney being the most frequent site involved. We report a case of sporadic AML in the hard palate of a 52-year-old male, an extremely unusual location for this tumour. Differentiation from other benign and malignant oral mesenchymal lesions depends on recognition of the three histologic components, and immunohistochemical techniques may be helpful. AML occurring in the head and neck do not express HMB-45, an antibody that identifies immature melanosomes, conversely to the usual immunopositivity shown in AMLs from kidney and liver, suggesting that there are differences among them. A wide surgical excision is considered curative, as this tumour usually behaves in a benign fashion.