Oral leiomyosarcoma. Report of a case and review of the literature pertaining to smooth-muscle tumors of the oral cavity.

Primary malignant smooth-muscle tumors of the mouth have rarely been reported. A review of the literature has yielded only eleven cases; to these, a new case is added. The age, sex, and site distribution of oral leiomyosarcomas are compared with those of oral leiomyomas.     

Primary gingival leiomyosarcoma. A clinicopathological study of 1 case with prolonged survival

BACKGROUND: Leiomyosarcoma is a relatively uncommon mesenchymal tumor that exhibits smooth-muscle differentiation. Only 3 to 10% of leiomyosarcomas arise in the head and neck, the nose and paranasal sinuses, skin and subcutaneous tissue and cervical esophagus being the most common localizations. Most leiomyosarcomas involving the oral tissues primarily affect the maxillary sinus, the maxillary or mandibular bone. A review of the English-language literature since 1908 revealed 30 reported cases of primary leiomyosarcoma of the oral mucosa and soft tissues. MATERIAL AND METHODS: We report on a case of gingival leiomyosarcoma, arising in a 31-year-old female and involving the upper alveolar mucosa. Following the diagnosis of malignant neoplasm on frozen sections and an en-block resection, the tumour was formalin-fixed and paraffin embedded for histological and immunohistochemical examination. RESULTS: Microscopically, the tumor was composed of interlacing fascicles of spindle-shaped cells with elongated, blunt-ended nuclei and eosinophilic cytoplasm, containing PAS-positive granules. Mitoses, both typical and atypical, and scattered necrotic foci were present. Consistent desmin, muscle specific and alpha-smooth muscle-specific, and vimentin immunoreactivity was demonstrated in the tumor cells. The patient is alive and free of disease at a 7-year follow-up. CONCLUSIONS: Intra-oral leiomyosarcomas are exceptionally rare. Accurate diagnosis and treatment is largely based on the careful search of clinical signs indicative of malignancy (e.g., neoplastic bone destruction, wide invasion of adjacent tissues) and intra-operative (frozen sections) examination of the lesion. Though the case reported herein showed an attenuated clinical behavior, prolonged follow-up is mandatory in view of possible tumor relapse.     

Primary gingival malignant melanoma. Report of 3 cases

BACKGROUND: Malignant melanoma is rare in the oral cavity and accounts for less than 1% of all melanomas. Nevertheless, the disease can be fatal, and early diagnosis and treatment may improve prognosis dramatically. The purpose of this paper is to report 3 new cases of primary malignant melanoma of the oral cavity arising in the gingiva, and to review the literature regarding intraoral melanoma. METHODS: Three cases are presented. One case was in the right mandibular molar area; the second in the right maxillary canine-premolar area; and the third in the left mandibular canine-premolar region. All patients were treated surgically, with postoperative radiotherapy. RESULTS: The first patient lived for 2 years and the second for 3 years before distant metastases were diagnosed from which they subsequently died. The third patient was lost from follow-up after 18 months. CONCLUSION: Primary oral malignant melanoma is a deadly disease. Early suspicion of this disease will allow prompt treatment and increase the prognosis for these patients.     

Leiomyosarcoma of the base of the tongue treated with radiotherapy: a case report

Soft tissue sarcomas of the oral cavity are uncommon malignancies; those of smooth-muscle origin are extremely rare. The world literature reveals about 80 leiomyomas and 26 cases of leiomyosarcoma. Only 4 of the cases with leiomyosarcoma occurred in the tongue. This report of leiomyosarcoma of the base and dorsum of the tongue adds 1 case which was primarily treated with irradiation and achieved and excellent response. A 1.5-year follow-up after radiation-therapy is included and a review of the literature is presented.     

Peripheral osteoma of the oral and maxillofacial region: a study of 35 new cases.

PURPOSE: The purpose of this article was to present 35 new cases of peripheral osteoma of the oral and maxillofacial region with an analysis of the literature. PATIENTS AND METHODS: We performed a search of our files for the past 5 years for peripheral osteoma of the oral and maxillofacial region. The criteria used to diagnose osteoma included the radiographic and histologic features. RESULTS: The 35 patients, which included 23 males (65%) and 12 females (35%), ranged in age from 14 to 58 years, with a mean age of 29.4 years. Most of the osteomas were located in frontal bone (28.57%), mandible (22.85%), and maxilla (14.28%). CONCLUSION: Peripheral osteomas of the jaw bones are uncommon, and accordingly, patients with osteoma should be evaluated for Gardner's syndrome. In addition, it is appropriate to provide both clinical and radiographic follow-up after surgical excision of a peripheral osteoma.     

Primary malignant melanoma of the oral mucosa.

PURPOSE: To present our experience on the epidemiology, clinical features, management, and survival of patients with oral malignant melanoma. PATIENTS AND METHODS: Records of patients with a histologic diagnosis of primary oral mucosal malignant melanoma seen over a 23-year period were retrospectively reviewed. RESULTS: There were 6 females and 2 males, ranging in age from 18 to 60 years; 4 cases in the maxilla, 2 in the mandible, 1 on the lower lip, and 1 on the buccal mucosa. Local recurrences developed in 2 patients who eventually died with clinical metastatic cervical nodal disease. Follow-up ranged from 6 months to 16 years 3 months. Surgery was the only treatment available. CONCLUSION: Primary oral mucosal melanoma is rare, with a 3 to 1 female to male ratio and an average age of 41.7 years at presentation. It is most common in the maxilla and has poor prognosis despite apparent adequate local surgical control.     

Clinicopathologic characterization of oral angioleiomyomas

OBJECTIVE: The purpose of this study was to better define the clinicopathologic features of oral angioleiomyomas. STUDY DESIGN: A retrospective search was performed for angioleiomyomas among all smooth muscle tumors accessioned from 1963 to 2001 in an oral pathology service. Twelve lesions met histopathologic criteria for inclusion in the study and were combined with 97 additional cases identified from the English language literature, yielding 109 cases for evaluation. RESULTS: The mean age of the patients with oral angioleiomyomas was 45.0 years, with a 1.43:1 male to female predilection. The most frequently reported site was the lip, in 48.6% of patients, followed by the palate (21.1%), buccal mucosa and tongue (each 9.2%), mandible (8.3%), and buccal sulcus, labial sulcus, floor of mouth, and gingiva (each 0.9%). Most mucosal lesions varied in size from a few millimeters to 2 cm, with most central lesions of the mandible measuring greater than 2 cm. Although angioleiomyomas are vascular lesions, only 55.9% of cases appeared red, blue, or purple; the remainder were gray, white, or the color of normal mucosa. Tumors were typically described as painless and manifested a low growth rate. All lesions were well circumscribed and composed of numerous vascular spaces surrounded by thick smooth muscle walls. CONCLUSION: Oral angioleiomyomas are benign smooth muscle tumors with a limited degree of morbidity. Careful histologic inspection is necessary to distinguish these lesions from their malignant counterpart, the leiomyosarcoma. Surgical excision is the treatment of choice, and recurrence is rarely encountered.     

Osteosarcoma of the jaw. The Chaim Sheba Medical Center experience

OBJECTIVE: The purpose of this article is to present 14 cases of osteosarcoma of the jaw treated at our medical center from 1989 to 1998. These cases are discussed in the light of a comprehensive review of 774 cases reported in the English literature over the past 3 decades. Differences between osteosarcoma of the jaws and osteosarcoma of the long bones are examined. SUBJECTS AND METHODS: The patients ranged in age from 8 to 78 years, the mean age being 33 years. Each patient had a histopathologically established diagnosis of osteosarcoma of the jaw. Records were reviewed for epidemiologic data, treatment modalities, and survival. RESULTS: Of the 14 patients, 6 (42%) had tumors in the mandible and 8 (58%) had tumors in the maxilla. Of the mandibular tumors, 5 occurred in the body of the mandible; all maxillary tumors originated in the alveolar ridge and involved the maxillary sinus. The chief complaint was an intraoral or extraoral painless swelling. Histopathologic types included chondroblastic, osteoblastic, fibroblastic, and malignant fibrous histiocytoma-like. Pathologic grade was determined to be high (3 or 4) in 13 cases and low (1) in only 1 mandibular case. All patients underwent surgical resection and immediate reconstruction. Adjuvant therapy included postoperative radiation (5 patients), postoperative chemotherapy (2 patients), and preoperative chemotherapy and postoperative radiation (1 patient). CONCLUSIONS: The results of the present study support the literature indicating that osteosarcoma of the jaw differs from osteosarcoma of the long bones in its biological behavior even though they have the same histologic appearance. Because of differences in tumor characteristics, the introduction of chemotherapy did not dramatically alter the prognosis of osteosarcoma of the jaw. Early diagnosis and radical surgery are the keys to high survival rates.     

Primary oral leiomyosarcoma: a retrospective clinical analysis of 20 cases

Oral Diseases (2010) 16 , 198–203 Purpose: As a review and clinical analysis of primary oral leiomyosarcoma (LMS) cases in West China stomatology Hospital in the past 37 years, this study provides demographic, therapeutic and prognostic information of this rare tumor. Patients and methods: In our study, 20 cases of primary oral LMS treated between 1972 and 2008 in West China Stomatology Hospital were analyzed retrospectively. A thorough review of clinical records was carried out and potential indicators of survival were analyzed. Results: The most common symptom of oral LMS presented as a painless mass. The median age of patients was 37 years, and the peak incidence age of this tumor was in the 2nd and 5th decades. There was no predilection of gender, and the male‐to‐female ratio was 11:9. The most frequently occurring site of oral leiomysarcoma was the jawbones. The prognosis of this tumor was poor as a result of the high local recurrence and the estimated 2 year survival was 17.6%. The bony involvement and method of therapy was observed to have an influence on the prognosis and survival of this tumor (P < 0.05). Conclusion: There was a predilection site of jawbones for oral LMS, and bony involvement was a potential indicator suggesting a poorer prognosis. The recommended method of therapy on this tumor was aggressive, radical surgical resection; however, adjuvant radiotherapy and chemotherapy may also have a beneficial effect.     

The surgical management of a leiomyosarcoma of the submandibular gland in a 95-year-old patient

Primary sarcomas of the major salivary glands are rare and appear to originate from undifferentiated pluripotential mesenchymal cells. They must be distinguished from malignant supporting tissue neoplasms that secondarily involve the glands by direct extension or metastasis. Multidisciplinary management of head and neck soft tissue sarcomas is still controversial. We report a case of leiomyosarcoma of the submandibular gland in a 95-year-old man who was treated with excision of the right submandibular gland, extended to the surrounding tissues, without neck dissection. The patient tolerated the treatment well. Twenty-four months after surgery, the patient was doing well without any evidence of locoregional or distant disease. Surgery is the cornerstone of the management of leiomyosarcomas of the salivary glands. Wide surgical excision with histologically proven tumor-free margins was an appropriate treatment that may guarantee prolonged survival.     

Osteolipoma: a review of the literature and a rare case report

Objectives

Lipomas are the most common benign mesenchymal tumors of soft tissue. According to previous studies, 1–4% of the cases has been observed in the oral cavity. A histological variant of lipoma featuring bone formation is called osteolipoma and has been very rarely observed (less than 1% of the total). In order to make a meaningful addition to this rare knowledge base, our study aims to provide a literature review and to report an additional case of osteolipoma.

Methods

An electronic search in the PubMed database with the keyword “osteolipoma” was conducted. Among 69 search results, only the cases of osteolipoma located in the “oral cavity” were included in this study. The findings of the previously reported 20 cases (in English) of osteolipoma of the oral cavity were organized in a table along with a new case of osteolipoma located in the mandibular buccal vestibule with radiological and histopathological findings provided by the authors.

Results

Osteolipoma affects both sexes and usually emerges in middle-aged or elderly patients with a long history of slow progression. Different imaging techniques may be utilized in the radiographic evaluation.

Conclusion

Differential diagnosis includes a wide range of lesions; therefore, the clinical and radiographic evaluation should be confirmed by histopathological examination. The suggested treatment is complete surgical excision and follow-up, and the prognosis is generally good.

     

Odontogenic ghost cell tumours. The neoplastic form of calcifying odontogenic cyst

The tumourous form of the calcifying odontogenic cyst is a rare entity and only a small number cases have been described in the medical literature published in English. Our report concerns a 66-year-old patient with a tumourous mass in the maxilla, with clear COC histological features, in addition to an infiltrative growth pattern of the epithelial islands and ghost cells invading the subjacent stroma, oral mucosa and peripheral maxillary bone. Cytoplasmic and nuclear pleomorphism and atypical mitoses were rarely found. An aggressive surgical approach produced a satisfactory result. We have reviewed the literature available in English and compared it with our case.     

Oral leiomyosarcomas: report of two cases with immunohistochemical profile

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-muscle-specific 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.     

Metastatic tumors to the jawbones: analysis of 390 cases

A review of the English language literature revealed 390 well-documented cases of metastatic lesions to the jawbones. Most metastatic lesions were diagnosed in patients in their fifth to seventh decade. The primary site differed between the genders: for women, it was the breast followed by the adrenal, colorectum, female genital organs and thyroid; for men, it was the lung, followed by the prostate, kidney, bone and adrenal. The most common location of the metastatic tumors was the mandible, with the molar area the most frequent site involved. In about 30% of cases the oral lesion was the first sign of the malignant disease. The present data are compared with those of metastatic tumors to the oral mucosa and a view on the possible pathogenesis is presented.     

Malignant Solitary Fibrous Tumor of the Scalp

Solitary fibrous tumors are an uncommon slow growing benign neoplasm originally described as a pleural neoplasm but can also be found in the lung, mediastinum, peritoneum, or any other sites including the head and neck. Malignant solitary fibrous tumors (MSFT) are extremely rare and only few cases have been published in the literature. There have been 19 cases reported of MSFT in the head and neck, but there are no reports of MSFT located within the scalp in the English language literature. We present a case of MSFT arising in the scalp and describe our experience with the clinical presentation, surgical management, and outcome in this pathological condition.     

A peculiar site of leiomyosarcoma: the tongue tip--report of a case

Primary leiomyosarcoma of the tongue is an exceedingly rare malignant mesenchymal tumour, which is hardly ever addressed in the differential diagnosis of a tongue lesion. Only 7 cases of this tumour have been documented in English-language medical literature. This study reports an additional case of primary leiomyosarcoma of the tongue in a 54-year-old woman, presenting with a foreign body sensation at the tip of the tongue with a rapidly and progressively enlarging ulcerative nodule. The patient received tumour excision with a 1-year follow-up without evidence of local recurrence or distant metastasis. Leiomyosarcoma in this unusual site seems to have better prognosis than elsewhere, although the number of cases are too small to draw a conclusion. This study further reviews the literature review and discusses the clinico-pathological presentation of this peculiar rare tongue lesion.     

Primary leiomyosarcoma of the mandible in a 7-year-old girl: report of a case and review of the literature

Leiomyosarcoma is a malignant neoplasm of smooth muscle origin that manifests itself uncommonly in the oral cavity because of the paucity of smooth muscle in that location. To the best of our knowledge, only 10 cases of leiomyosarcoma primary to the jawbones have been reported in the English language literature. We report the first pediatric case of leiomyosarcoma arising from the mandible. Facial asymmetry and swelling were accompanied by a rapidly growing exophytic soft tissue mass that caused buccal displacement of the mandibular left permanent first molar. The lesion, observed radiographically as an extensive ill-defined area of osteolytic alveolar destruction, perforated the lingual cortex, displaced the inferior alveolar nerve canal inferiorly, and produced a "floating-in-air" appearance of the first molar. Diagnosis of leiomyosarcoma was made after initial incisional biopsy of the lesion. A 5-cm segmental mandibulectomy and supraomohyoid neck dissection were followed by reconstruction with a dynamic mandibular reconstruction plate and placement of a multidimensional mandibular distraction device in a transport rectangle of bone to promote bifocal distraction osteogenesis. Forty millimeters of distraction (the technical limit of the device) were performed; this was followed by terminal iliac crest bone grafting. Seventeen months after the definitive surgical procedure, the patient remains free of disease.     

Oral vascular leiomyoma: review of the literature and report of two cases

A bstract — A review of 78 cases of oral leiomyoma from the literature shows that oral leiomyomas differ from those elsewhere in the body and they are usually vascular in type. Oral leiomyosarcomas occur only one-fifth as commonly as the leiomyomas. The histologic criteria for the differentiation of leiomyosarcoma are equivocal and so accurate prediction of the behaviour of such lesions is difficult.
A histopathologic investigation of two cases was undertaken to establish the usefulness of certain special stains, the presence or absence of neoplastic features and to estimate behaviour of these tumours. This showed that a connective tissue stain and the demonstration of myofibrils within the smooth muscle cells were minimal requirements for accurate diagnosis. The two lesions were well defined and contained well differentiated leiomyoblasts with no mitotic figures. A prediction of benign behaviour was borne out by clinical follow-up.     

Leiomyosarcoma of the tongue: a case report

Primary leiomyosarcomas arising from within the tongue are extremely rare. Only eight cases have been previously reported on Medline to our knowledge. We describe a case of a 46-year-old woman who presented with a lesion rapidly increasing in size on the lateral aspect of her tongue, which was excised. We present a completed-5-year follow-up.     

Primary oral leiomyosarcoma: A systematic review and update

The aim of this systematic review was to address the clinicopathologic inconsistencies noted with primary oral leiomyosarcoma in the literature by amassing the available data published into a comprehensive analysis. Eligibility criteria included publications of cases with dedicated immunohistochemical work‐up along with radiographs to evaluate location. Based on these criteria, the systematic review compiled 29 cases. Four primary location sites were identified in the reported cases: soft tissue presentation only, soft tissue with bony involvement, bony involvement only, and bony involvement with a soft tissue component. The majority of primary oral leiomyosarcoma cases reviewed showed a soft tissue predilection, which is in contrast to prior reports of jawbones being the most common site. In addition, there was an improved 5‐year survival rate for primary oral leiomyosarcoma limited to the oral cavity and gnathic bones without extension into paranasal sinuses.