Primary Leiomyosarcoma of the Mandibular Alveolar Mucosa of a 12-Year-Old Child from Ethiopia: A Case Report (original) (raw)
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Oral leiomyosarcomas: report of two cases with immunohistochemical profile
Oral Surgery Oral Medicine Oral Pathology Oral Radiology and Endodontology, 2007
Leiomyosarcoma of the oral cavity is a very rare tumor associated with aggressive clinical behavior and low survival. In this paper, we report 2 cases of leiomyosarcoma, affecting the gingival mucosa of a 54-year-old female and the maxillary bone of a 63-year-old male. Histologically, the tumors were composed of variably oriented fascicles of spindle-shaped cells with cigar-shaped nuclei and eosinophilic cytoplasm. The lesions were treated by surgical resection. Immunoreactivity to anti-vimentin, anti-smooth muscle actin, anti-desmin, anti-laminin, and anti-musclespecific actin antibodies were found; conversely, the tumor cells were negative for anti-S100 and AE1/AE3 proteins. This report emphasizes the role of immunohistochemical study for correct diagnosis of leiomyosarcoma. (Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2007;104:e50-e55)
Leiomyosarcoma of mandible: A case report and review of literature
groups, one wherein each tumor type is characterized by a unique simple recurrent genetic abnormality such as a chromosomal translocation and the other in which highly complex genetic abnormalities are present (1). LMS belong to the latter group and are malignant neoplasms of smooth muscle, which most frequently occur in the uterus or retroperitoneum but can occur throughout the body (1). Women are more affected than men because of the uterine association. The prognosis is poor, with a high percentage of recurrence or metastasis. The most common sites of metastasis include the lungs, bone, brain and the lymph node. This neoplasm is an extremely rare mesenchymal lesion in the oral cavity, with unusual bone location because of paucity of smooth muscle in that site (2). The immunohistochemical analysis for cytoskeletal proteins such as actin, vimentin, desmin, Ki-67 and ultrastructural examination has been widely used for the diagnosis of LMS (3, 4). The aim of this article is to describe a rare case of LMS of the mandible with immunohistochemical analysis that was useful in making the final diagnosis. CASE REPORT A 65-year-old woman reported to our institute with chief complaint of pain and swelling on right facial region since 5 years. Patient was apparently all right 5 years ago, and then she noticed a small swelling on same region which gradually increased to present size. There were no associated symptoms accept intermittent dull pain. On examination a growth was observed with multiple nodules extending from corner of the mouth to angle of mandible measuring about 10X7 cm in size (Fig 1). Intraorally the lesion appeared as homogenous mass over right vestibular area extending from central incisor to molar area with central necrosis at body region. On palpation swelling was soft to firm in consistency with no tenderness and skin was attached to underlying structure. No lymph nodes involvement was found. Otherwise patient was healthy and all hematological and biochemical investigations were under normal limits.
2013
Purpose: Leiomyosarcoma (LMS) rarely occurs in the head and neck region. These tumors present with a wide range of clinical features, so the diagnosis is predicated on conventional microscopic findings coupled with immunohistochemical analysis. Patients and Methods: Clinical and histologic data of 7 patients with LMS of the head and neck were recorded retrospectively. In addition to routine immunohistochemistry, staining for cell cycle regulator proteins p16 and p21 was performed. Results: Five LMSs (4 intraoral, 1 dermal cheek) occurred primarily in the oral and perioral region. Two LMSs (parietal and sinonasal) were diagnosed as metastases originating from the uterus and pelvis. Treatment of the primary LMSs consisted of radical tumor resection with clear margins. Distant metastases from LMSs were irradiated or excised as palliative treatment. Three of 5 patients (60%) with primarily excised LMS developed recurrence after an average of 7 months, with lung metastases occurring after 17 months. In 1 patient, cervical lymph node metastases were detected after 10 months. Of all patients, 5 died after an average survival period of 2.4 years. The mean survival period of the 5 patients with primary LMS of the head and neck was 3.3 years. All tumors were positive for vimentin and a-smooth muscle actin, with 57% of tumors showing positive nuclear expression of p16 and 71% of p21. Lack of p16 nuclear expression was associated with a shorter mean survival time (1.3 vs 4.3 yr for p16 positivity). Conclusion: Lung and cervical lymph node metastases often occur in LMS of the head and neck. Presurgical staging, including gynecologic examination, whole-body computed tomography, and sometimes positron-emission or computed tomography, to rule out LMS metastasis is mandatory. Surgical resection of the tumor should be given top priority. Lack of p16 reactivity may have a prognostic value for LMS because it was related to a trend toward poorer survival.
Oral Oncology, 2002
Leiomyosarcoma of the oral cavity is a very rare tumor that is associated with aggressive clinical behavior and low survival. In this paper, we report two new cases of leiomyosarcoma affecting the mandibular gingiva and mandible of a 35-year-old male and the mandible of a 51-year-old female. Given the difficulty in the histopathologic discrimination between benign and malignant smooth muscle tumors and the absence of reliable histologic parameters for prognostication of leiomyosarcomas, we evaluated the diagnostic and prognostic value of various immunohistochemical and molecular markers. By means of immunohistochemistry and quantitative real-time PCR analysis, we detected protein expression of PCNA, bcl-2, CDK4, p53 and MDM2 in both our cases and MDM2 amplification in our second case. The literature, pertinent to oral leiomyosarcoma and to molecular analysis of smooth muscle tumors, is reviewed. #
Oral Oncology, 2000
Leiomyosarcoma is a relatively uncommon mesenchymal tumor that exhibits smooth-muscle dierentiation. We report a new case of leiomyosarcoma involving the tongue of a 67-year-old male. Histologically, the tumor was composed of variably oriented fascicles of spindle-shaped cells with`cigar-shaped' nuclei and eosinophilic cytoplasm, containing occasional PAS-positive granules. Atypical mitotic ®gures and necrotic foci were frequently detected. Consistent desmin, a-smooth muscle-speci®c and sarcomeric actin, and vimentin immunoreactivity was demonstrated in the tumor cells, whereas cytokeratins, CD 30, CD 31, CD 34, CD 45, CD 68, EMA, GFAP, HMB 45 and S-100 protein were negative. The patient underwent wide surgical excision of the tumor and is alive and disease-free at a 5-year follow-up. This report emphasizes the diculties in the dierential diagnosis of these uncommon tumors in an intra-oral location. #
Leiomyosarcomas of the oral cavity. Report of four cases and review of the literature
Journal of Cranio-Maxillofacial Surgery, 1993
SUMMAR Y. Leiomyosarcomas (LMS) make up 7 % of all soft tissue sarcomas. In the oral cavity, the LMS is rare due to the paucity of smooth muscle in that region. Four cases of intraoral LMS are reported, plus 34 cases found in the literature reviewed. There were 24 males and 14 females. The age range at presentation was 10 months to 88 years with no predilection for any particular age group. The commonest presenting symptom was a mass. The intraoral LMS occurred most commonly in the jaws (59 % of cases). The mass was painful in 61% of cases. Followup of the case reports was assessed. Recurrence occurred in 36 % of cases. Distant metastases occurred in 39 % of cases, most commonly to the lungs. Cervical nodal metastasis was reported in 15 % of cases. The 5-year survival rate determined on cases with adequate follow-up (n = 13) was 23 % free of disease, 8 % alive with disease and 69 % who died of disease. In comparison with stage I and stage I1 tongue squamous cell carcinomas, the intraoral LNIS is very aggressive. It is best treated surgically, early and aggressively.
Lingual Leiomyosarcoma: A Histopathological Case Report
Cureus
Leiomyosarcomas are rare malignant mesenchymal neoplasms originating from smooth muscle cells. Although leiomyosarcoma is commonly located in the female reproductive system, gastrointestinal tract, and subcutaneous tissues, it is a rare entry in the head and neck area, probably due to the scarcity of smooth muscle tissue in this topographical region. Herein we present a histopathological case report of a 60-yearold male with a slow-growing painless mass on the middle third of the right lateral lingual margin, with focal ulceration measuring 1x1.5cm. After gross excision, histopathology revealed pleomorphic spindle cells, some with bizarre nuclei and abundant pathological mitotic activity with a tendency to grow in a fascicular pattern. As the patient had the demographic characteristic and risk factors for oral cavity squamous cell carcinoma, a spindle-shaped variety (sarcomatoid) variety was suspected, and immunohistochemistry with a broad set of antibodies was used to prove the histogenetic group of the tumor. As the tumor was pancytokeratin and desmin negative, focally positive for caldesmon, and positive for smooth muscle actin, the diagnosis of pleomorphic leiomyosarcoma of the tongue was established.
Leiomyosarcoma in the mandible
Medicine, 2016
Leiomyosarcoma (LMS) is a malignancy which very rarely occurs in maxillofacial location, and the course of the disease is not very characteristic. In this case report, we present a 58-year-old female patient with a painless tumor of the left angle of the mandible causing slight asymmetry of the face. She also reported that she observed deterioration in fitting of the lower denture in the oral cavity for several months, which she had used successfully for 5 years. On the basis of clinical tests, histopatological examination, and imaging (CT, MRI, ultrasound, pantomography), the patient was diagnosed with primary malignant leiomyosarcoma (LMS) of the mandibular corpus and ramus on the left side. The patient received combined surgical and oncological treatment. The first stage was a surgery, and then adjuvant radiotherapy was applied on the site of the resected tumor-a total dose of 60 Gy in 35 fractions. The patient's postoperative course was uneventful. She also underwent adjuvant therapy well. In the period of 3-year follow-up, no signs of recurrence were observed. The findings may extend our knowledge and experiences in the treatment of leiomvosarcoma in the craniofacial area. Abbreviations: BAC = fine needle aspiration biopsy, CT = computer tomography, IHC = immunohistochemical, Ki-67 = Ki-67 protein, LMS = leiomyosarcoma, MRI = magnetic resonance imaging, RMS = rhabdomyosarcoma, S100 = S-100 protein, SMA = smooth muscle actin.
Leiomyosarcoma of Mandible: A Diagnostic Dilemma; Case Report and Review of Literature
Indian Journal of Otolaryngology and Head & Neck Surgery, 2019
Leiomyosarcoma and its pleomorphic variant are rare entities in the head and neck region. Since they usually present as slow growing, discrete firm, and non-ulcerated painless mass, they seem to be deceptively benign and are thus misdiagnosed. Histopathological and immunohistochemical studies are the ways of getting a definitive diagnosis. Till date surgery has been the primary treatment but effectiveness of radiotherapy/chemotherapy is still questionable. Here, we describe a case of leiomyosarcoma in mandible along with discussion about the ways of diagnosis, its differentiation with its pleomorphic variant and their managements.