Angioleiomyoma Research Articles

Ultrasound and magnetic resonance imaging (MRI) features of angioleiomyoma.

Vascular leiomyoma or angioleiomyoma is a rare benign tumor which belongs to the group of benign smooth muscle tumors. They are commonly located at the extremities, usually presented as a painful solitary lesion in the subcutaneous tissue. To date, very few reports describe its characteristics from an imaging point of view, especially their ultrasound characteristics, making it difficult to do a pre-surgical diagnosis. Our purpose is to describe the clinical, pathologic and imaging features of angioleiomyoma [ultrasound, Doppler ultrasound and magnetic resonance imaging (MRI) findings]. We retrospectively reviewed from the pathology database from Hospital Universitario Fundación Alcorcón, Madrid, Spain, the clinical histories of 139 patients who had surgical excision and histologic diagnosis of angioleiomyoma during the last 17 years, from May 31st 2006 to April 17th 2023. Of those patients, we focused on 50 who had soft tissue angioleiomyoma with imaging study [ultrasonography (US) and/or MRI] performed in our institution, making a descriptive cross-sectional study. In our series, a very characteristic ultrasonographic and US Doppler pattern was found. It consists in the presence of a well defined, homogeneous and highly vascularized soft tissue tumor, sometimes with the presence of a feeding vessel. When the described US features appear in a mobile and elastic subcutaneous slow growing tumor on an extremity, the diagnosis of angioleiomyoma should be considered as highly probable.

Angiomyolipomatous Lesions of the Nasal Cavity (Sinonasal Angioleiomyoma with Adipocytic Differentiation): A Multi-Institutional Immunohistochemical and Molecular Study.

Mesenchymal neoplasms composed of vascular, smooth muscle, and adipocytic components are uncommon in the nasal cavity. While angioleiomyoma (AL) is a smooth muscle tumor in the Head & Neck WHO classification, it is considered of pericytic origin in the Skin as well as Soft Tissue and Bone classifications. For nasal AL with an adipocytic component, the terms AL with adipocytic differentiation and angiomyolipoma (AML) have been applied, among others. AML is a type of perivascular epithelioid cell tumor (PEComa), most often arising in the kidney, sometimes associated with the tuberous sclerosis complex (TSC). It is uncertain whether nasal cavity AML and AL are best considered hamartomas or neoplasms, as their genetics are largely unexplored. We performed a multi-institutional retrospective study of nasal cavity mesenchymal lesions. Patient demographics, clinical histories, and histologic and immunohistochemical findings were collected. DNA and RNA were extracted from formalin-fixed, paraffin-embedded tissue and analyzed by SNP-based chromosomal microarray, targeted RNA fusion sequencing, and whole-exome sequencing. Fifteen lesions (3-42mm) were identified, predominantly in male (87%) patients with a median age of 60. Patients typically presented with obstructive symptoms, and none had a history of TSC. One AL was a recurrence from six years prior; 11 cases showed no recurrence (median 4.7 years, range: 0.88-12.4). Morphologically, 11 AML contained 30-80% smooth muscle, 10-25% vasculature, and 2-60% adipose tissue, while four AL contained 70-80% smooth muscle and 20-30% vasculature. Other histologic observations included ulceration, thrombosis, inflammation, myxoid change, senescent nuclei, and extramedullary hematopoiesis; no well-developed epithelioid cell morphology was identified. Immunohistochemically, all cases were positive for smooth muscle markers (actin, desmin, and/or caldesmon) and negative for melanocytic markers. Molecular analysis revealed loss of 3p and 11q in a single AML. No other known pathogenic copy number or molecular alterations were seen, including in TSC1/2, TFE3, or NOTCH2. Nasal cavity AML lacks morphologic, immunophenotypic, and genetic features of PEComa family AML. The significant histologic overlap between nasal AML and AL without distinguishing molecular features in either entity suggests "sinonasal angioleiomyoma with adipocytic differentiation" may be the most appropriate terminology for hybrid vascular and smooth muscle lesions containing adipocytic components.

Angiomyolipomatous Lesions of the Nasal Cavity (Sinonasal Angioleiomyoma with Adipocytic Differentiation): A Multi-Institutional Immunohistochemical and Molecular Study.

Mesenchymal neoplasms composed of vascular, smooth muscle, and adipocytic components are uncommon in the nasal cavity. While angioleiomyoma (AL) is a smooth muscle tumor in the Head & Neck WHO classification, it is considered of pericytic origin in the Skin as well as Soft Tissue and Bone classifications. For nasal AL with an adipocytic component, the terms AL with adipocytic differentiation and angiomyolipoma (AML) have been applied, among others. AML is a type of perivascular epithelioid cell tumor (PEComa), most often arising in the kidney, sometimes associated with the tuberous sclerosis complex (TSC). It is uncertain whether nasal cavity AML and AL are best considered hamartomas or neoplasms, as their genetics are largely unexplored. We performed a multi-institutional retrospective study of nasal cavity mesenchymal lesions. Patient demographics, clinical histories, and histologic and immunohistochemical findings were collected. DNA and RNA were extracted from formalin-fixed, paraffin-embedded tissue and analyzed by SNP-based chromosomal microarray, targeted RNA fusion sequencing, and whole-exome sequencing. Fifteen lesions (3 to 42 mm) were identified predominantly in male (87%) patients with a median age of 60. Patients typically presented with obstructive symptoms, and none had a history of TSC. One AL was a recurrence from six years prior; 11 cases showed no recurrence (median 4.7 years, range: 0.88-12.4). Morphologically, 11 AMLs contained 30-80% smooth muscle, 10-25% vasculature, and 2-60% adipose tissue, while four ALs contained 70-80% smooth muscle and 20-30% vasculature. Other histologic observations included surface ulceration, vascular thrombosis, chronic inflammation, and myxoid change; no well-developed epithelioid cell morphology was identified. Immunohistochemically, all cases were positive for smooth muscle markers (actin and/or desmin) and negative for melanocytic markers. Molecular analysis revealed loss of 3p and 11q in a single AML. No other known pathogenic copy number or molecular alterations were seen, including in TSC1/2, TFE3, or NOTCH2. Nasal cavity AML lacks morphologic, immunophenotypic, and genetic features of PEComa family AMLs. The significant histologic overlap between nasal AML and AL without distinguishing molecular features in either entity suggests "sinonasal angioleiomyoma with adipocytic differentiation" may be the most appropriate terminology for hybrid vascular and smooth muscle lesions containing adipocytic components.

Leiomyoma of the Nasal Septum – A Case Report

Leiomyomas of the nasal cavity are rare and benign tumour of vascular smooth muscle origin that uncommonly arise from the nasal septum. In the nasal cavity they contribute less than 1% of all human leiomyoma. Leiomyomas are more commonly found in female genital tract (uterus), gastrointestinal tract and subcutaneous tissue. They are classified in three groups: leiomyoma, angiomyoma and epithelioid leiomyoma. Here, we report a clinical case of a rare vascular leiomyoma in the nasal septum in a 32-year-old man. The treatment of choice was surgical excision. A brief review of the literature, treatment and aetiological variations are discussed. CBMJ 2024 July: vol. 13 no. 02 P: 274-276

A rare case of a vascular benign tumor in oral cavity – Case report

Leiomyomas are benign tumors arising from smooth muscle and can be classified as solid, epithelioid, and vascular leiomyoma (angioleiomyoma). Angioleiomyoma frequently occurs on the skin. Due to the lack of smooth muscle in the oral cavity, this tumor is rarely located in the mouth. This study aims to present a rare case of a 36-year-old woman with a painless submucosal nodule in the left cheek mucosa, measuring approximately 2 cm x 1 cm, with 5-years of evolution. Morphological and histochemical examination evidenced the proliferation of smooth muscle around the blood vessels of various calibers. The diagnosis of angioleiomyoma was confirmed, the tumor was surgically removed, and the patient had no further complications after 9-months of follow-up. The diagnosis of oral angioleiomyoma is challenging since this tumor is rare and can mimic or transform into malignancy. For this reason, histological examination in association with immunohistochemical is invaluable to establishing an accurate diagnosis and delivering suitable treatment.

Vascular Leiomyoma of oral cavity: A case report in young male patient

Abstract Vascular Leiomyoma of the oral cavity is an uncommon benign soft tissue neoplasia arising from the tunica media of the smooth muscle cells. We reported a rare case of Vascular Leiomyoma involving the retromolar trigone area of a young man. A 24-year-old male presented with a slowly growing mass in the retromolar area, which was associated with pain and difficulty in opening of mouth and eating and drinking. A punch biopsy was taken and sent for histopathological examination which revealed a benign angiomatous lesion. The mass was excised, and the adjoining area was curetted. Histopathological examination confirmed the diagnosis of Vascular Leiomyoma. The immunohistochemistry of cells shows: alpha-smooth muscle (+); vimentin (+), Desmin, actin (+), and S100 (−). Angioleiomyomas are benign lesions, but malignant transformation is a possibility so careful histologic inspection is necessary to distinguish these lesions from their malignant counterpart.

Angioleiomyoma of the Pterygopalatine Fossa: A Case Report.

Angioleiomyoma (ALM) is a benign neoplasm marked by the presence of blood vessels and smooth muscle cells, commonly located within the subcutaneous or deep dermal layers. Its manifestation in the pterygopalatine fossa is infrequent, thereby posing difficulties in its diagnosis and distinction from other benign tumors within the same anatomical site. In the present case study, an ALM originating in the right pterygopalatine fossa of a 44-year-old female patient was investigated. The patient underwent surgical intervention for the vascular smooth muscle tumor, and there has been no recurrence noted during the postoperative monitoring period.

Retroperitoneal Angioleiomyoma: An Extremely Rare Case Report

Angioleiomyoma (ALM) is a benign mesenchymal tumor arising from the smooth muscle cells of the vessel wall. Common sites include subcutaneous tissues of the lower extremities but can also be seen in unusual sites including the head-and-neck region, submandibular gland, uterus, ovary, and broad ligament. It is exceptionally rare in retroperitoneum. Local recurrence is extremely rare but known to occur. Imaging features are nonspecific. Differential diagnosis on the basis of radiological features is difficult. Definitive diagnosis requires histopathology examination. Angiomyolipoma and myopericytoma are considered close differentials on microscopy at retroperitoneal locations. Histopathological examination along with immunohistochemistry markers such as desmin and HMB-45 are required for definitive diagnosis. It is difficult to diagnose angioleiomoma preoperatively, considering its location, thereby we are tempted to present an extremely rare case of retroperitoneal ALM highlighting its diagnostic dilemma.

Ethmoidal Sinonasal Angioleiomyoma: A Rare Case With A Diagnostic Challenge

Sinonasal Angioleiomyomas are rare benign mesenchymal tumours consisting of well differentiated smooth muscles proliferation with vascular component that represent less than 1 percent of all the vascular leiomyomas. The most effective way of detecting sinonasal angioleiomyoma preoperatively is by computed tomography however the postoperative histopathological remains the gold standard in diagnosis of vascular leiomyomas. In this case report we present a case of 45 years old female with positive history of nasal obstruction, epistaxis, hyposmia and facial pain. Physical examination revealed whitish mass in both nasal cavities with decrease nasal patency in both nostrils. After endoscopic resection, the diagnosis of sinonasal angioleiomyoma was established based on histopathology. Since ethmoidal sinuses are relatively uncommon area for the sinonasal angioleiomyomas to occur therefore we suggest that one should keep them in mind when making differential diagnosis of nasal obstruction with epistaxis. It is also worth noting that this is the first reported case of sinonasal angioleiomyoma arising from the ethmoidal sinuses from Pakistan.

Angioleiomyoma of the Sinonasal Tract: A Systematic Review of an Uncommon Clinicopathological Entity.

Introduction Angioleiomyoma is a rare neoplasm that represents ∼ 0.2 % of all head and neck benign tumors and ∼ 2% of total cases of tumors of the sinonasal tract. It was once considered a possible subtype of leiomyoma, but, in the 2020 World Health Organization (WHO) classification of soft tissue tumors, it is accepted as a singular entity. Objective To systematically review the existing literature on angioleiomyoma in the light of the new classification of soft tissue tumors. Data Synthesis The present study was performed in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. A comprehensive search in the PubMed, Cochrane, Scopus, and Google Scholar databases was performed in January 2022. The search items included the following keywords: nasal angioleiomyoma OR sinonasal angioleiomyoma OR nasal vascular leiomyoma OR sinonasal vascular leiomyoma . A total of 87 patients were evaluated. He age of the patients in the studies ranged from 15 to 88 years (mean age at diagnosis: 55.6 years). The most common site of involvement was the nasal septum (28.4 %), followed by the inferior turbinate (22.5%). The most common symptom was nasal obstruction (66.7%), followed by epistaxis (47.1%). Surgical excision represented the main treatment, and there was recurrence of pathology in only 1 case (0.9%). Conclusion To our knowledge, only 87 cases of sinonasal-tract angioleiomyoma have been previously described. The results of the present review seem to confirm the rarity and the benign nature of this neoplasm, and they seem to confirm the necessity to improve the available data about sinonasal-tract angioleiomyoma.

Vascular Leiomyoma Arising From the Nasal Floor Misdiagnosed as a Nasolabial Cyst in an 74-Year-Old Patient

Leiomyoma is a benign tumor that originates from smooth muscle and can develop anywhere in the body where smooth muscle is present. However, leiomyoma arising in the head and neck region are very rare. We experienced a case of a 74-year-old male patient with a right nasal floor mass. The patient complained of bleeding from the right nasal cavity, nasal congestion, and discharge. On physical examination, a relatively smooth-surfaced, well-demarcated mass was observed in the right nasal floor. Preoperative imaging was interpreted as a right nasolabial cyst, and endoscopic excision was performed. Histologic examination confirmed it to be vascular leiomyoma. The patient was followed up for 6 months postoperatively and remains stable with no recurrence of symptoms. We report a case of vascular leiomyoma arising from the nasal floor that was mistaken for a nasolabial cyst in a 74-year-old patient.

Angioleiomyoma of parotid gland: a rare case report

Angioleiomyoma or vascular leiomyoma first described by Virchow is a very uncommon benign soft tissue tumor. Angioleiomyoma has been defined as frequently painful, benign subcutaneous or deep dermal tumor composed of mature smooth muscle bundles which are surrounded and interlaced by vascular channels. Only 8.5% cases arise in the head and neck. A 39 years old male presented with swelling right side face and upper neck past five years. The swelling was non tender, immobile and non-compressible. There was lateral pharyngeal wall bulge which was pushing the tonsil medially. The patient underwent total conservative parotidectomy and histopathology (HPE) came out to be angioleiomyoma. Angioleiomyoma is difficult to diagnose correctly from clinical manifestations and imaging alone, and a biopsy is mandatory to make the diagnosis.

Experience with endonasal endoscopic resection of non-angiofibroma sinonasal and orbital apex vascular tumours

Background: Endoscopic endonasal approach for the management of sinonasal inflammatory pathologies and tumours has been a widely accepted procedure and used for many years. The aim of the study is to assess effectiveness and safety of endonasal endoscopic resection in removal of sinonasal vascular tumours and to evaluate outcomes and clinical behaviour of different subtypes with review of the literature. Material and Methods: A retrospective review of the patients treated for sinonasal and orbital apex vascular tumour was performed. Patient’s demographics, surgical approaches, complications, histopathological results, and long-term outcomes were evaluated. Results: Twenty-two patient included in this study. The mean tumour size ranged from 6 to 100 mm (30.45 ± 22.7 mm). Histopathological examination revealed 8 (36%) capillary hemangioma, 6 (33.3%) cavernous hemangioma, 2 (12%) mixed hemangioma, and 2(12%) vascular leiomyomas. Three (13.6%) patients were diagnosed as glomangiopericytoma and remaining 1 (4.4%) was angiosarcoma. Only in 1 patient with recurrent glomangiopericytoma preoperative embolization were needed. Five patients had preoperative biopsy in office settings. The mean follow-up was 72.9 (± 53.71) months. The recurrence was observed in 3 (13.6%) patients. Conclusion: Endonasal endoscopic approach for sinonasal vascular tumours is a safe and reliable method for resection. Our study suggested location of the tumour is more important than the size to achieve complete resection. Long-term follow-ups are important to detect recurrences early even after macroscopically complete resections.

Benign Cutaneous Tumors

Tumors of the cutaneous surface may arise from the epidermis, dermis, or subcutaneous tissue or from any of the specialized cell types in the skin or its appendages. Broad categories include tumors derived from epithelial, melanocytic, or connective tissue structures. Within each location or cell type, lesions are classified as benign, malignant, or, in certain cases, premalignant. Benign epithelial tumors include tumors of the surface epidermis that form keratin, tumors of the epidermal appendages, and cysts of the skin. Melanocytic (pigment-forming) lesions are very common. One of the most frequently encountered forms is the nevus cell nevus. Tumors that are derived from connective tissue include fibromas, histiocytomas, lipomas, leiomyomas, and hemangiomas. This chapter provides an overview of each type of tumor, including sections on epithelial tumors, tumors of the epidermal appendages, familial tumor syndromes, melanocytic tumors, neural tumors, connective tissue tumors, vascular birthmarks, acquired vascular disorders, Kimura disease, lipoma, leiomyoma, and lymphangioma circumscriptum. The sections discuss various forms and their diagnosis, differential diagnosis, and treatment. Figures accompany the descriptions. This chapter contains 83 references, 26 highly rendered figures, and 5 MCQs.

Benign Cutaneous Tumors

Tumors of the cutaneous surface may arise from the epidermis, dermis, or subcutaneous tissue or from any of the specialized cell types in the skin or its appendages. Broad categories include tumors derived from epithelial, melanocytic, or connective tissue structures. Within each location or cell type, lesions are classified as benign, malignant, or, in certain cases, premalignant. Benign epithelial tumors include tumors of the surface epidermis that form keratin, tumors of the epidermal appendages, and cysts of the skin. Melanocytic (pigment-forming) lesions are very common. One of the most frequently encountered forms is the nevus cell nevus. Tumors that are derived from connective tissue include fibromas, histiocytomas, lipomas, leiomyomas, and hemangiomas. This chapter provides an overview of each type of tumor, including sections on epithelial tumors, tumors of the epidermal appendages, familial tumor syndromes, melanocytic tumors, neural tumors, connective tissue tumors, vascular birthmarks, acquired vascular disorders, Kimura disease, lipoma, leiomyoma, and lymphangioma circumscriptum. The sections discuss various forms and their diagnosis, differential diagnosis, and treatment. Figures accompany the descriptions. This chapter contains 83 references, 26 highly rendered figures, and 5 MCQs.

Benign Cutaneous Tumors

Tumors of the cutaneous surface may arise from the epidermis, dermis, or subcutaneous tissue or from any of the specialized cell types in the skin or its appendages. Broad categories include tumors derived from epithelial, melanocytic, or connective tissue structures. Within each location or cell type, lesions are classified as benign, malignant, or, in certain cases, premalignant. Benign epithelial tumors include tumors of the surface epidermis that form keratin, tumors of the epidermal appendages, and cysts of the skin. Melanocytic (pigment-forming) lesions are very common. One of the most frequently encountered forms is the nevus cell nevus. Tumors that are derived from connective tissue include fibromas, histiocytomas, lipomas, leiomyomas, and hemangiomas. This chapter provides an overview of each type of tumor, including sections on epithelial tumors, tumors of the epidermal appendages, familial tumor syndromes, melanocytic tumors, neural tumors, connective tissue tumors, vascular birthmarks, acquired vascular disorders, Kimura disease, lipoma, leiomyoma, and lymphangioma circumscriptum. The sections discuss various forms and their diagnosis, differential diagnosis, and treatment. Figures accompany the descriptions. This chapter contains 83 references, 26 highly rendered figures, and 5 MCQs.

Letter to the Editor Regarding “Academic Accomplishments of Female Neurosurgeons Currently in Practice in the United States”

Skull base leiomyomas (LMs) and angioleiomyomas (ALMs) are rare, and the understanding of this disease is limited. We present a systematic literature review of skull base LM and ALM and report a case of internal auditory canal (IAC) ALM.A systematic review was conducted following the PRISMA guidelines. PubMed and Embase were systematically queried for skull base LM and ALM, and Rayyan QCRI was used for the review. After applying exclusion criteria, individual articles were evaluated for quality control, data collection, and analysis. The presentation, management, and outcome of a 37-year-old man with a right-sided IAC ALM are described.Of 68 unique entries, 27 studies were included. Thirty-four cases of skull base LM (n = 6) or ALM (n = 28) were identified. Average age at presentation was 45.1 ± 14.5 years, and 52.9% of patients were male. Tumor diameter was 2.75 ± 1.6 cm, with headaches being the most reported symptom. Commonly reported locations were the cavernous sinus and the external auditory canal. Only 3 cases of IAC ALM met the criteria for this review. All tumors were treated with surgery, and gross total resection was achieved in 27 patients. Radiation was given in 3 cases with subtotal resection.Skull base LM and ALM are rare. Given the need for pathology, surgery has been the standard treatment for symptomatic skull base LM and ALM. It is important to understand the available data about this disease and consider it in the differential of skull base lesions.

Acral Calcified Angioleiomyoma Along with Radiological Features

Angioleiomyoma (ALM) is a rare benign, vascular smooth muscle tumor originating from the tunica media of the vessel wall. It typically arises in the cutaneous, subcutaneous tissue of the lower extremities in middle-aged women...

Cutaneous leiomyomas: a clinicpathological and epidemiological review

Cutaneous, superficial and or suprafascial leiomyoma are divided into three variants: piloleiomyomas (PL), angioleiomyomas (AL) and genital leiomyomas (GL) that include the vulvar, scrotal and areolar forms. This study set out to establish the clinical and histological characteristics and incidence of each variant, and any likely associations with internal neoplasms. A review was carried out of 255 cases of cutaneous leiomyomas diagnosed between 1982 and 2018 at the Pathology departments of three hospitals (Navarra and Alicante). Demographic, clinical, histological and immunohistochemical variables were described and compared. The incidence of PL in Navarra was 4.3 cases per million inhabitants a year, with another 20 cases of AL and 1.4 cases of GL. Cutaneous forms make up approximately 3.5% of the total leiomyomas. The population with PL suffered more frequently from breast cancer (OR?=?4.8; CI 95%: 1.3-17.4; p?=?0.006). Nipple leiomyomas are small, accompanied by localised pain, and are predominantly fascicular or solid, with very infrequent effect on the subcutaneous cellular tissue and scarce atypia. This makes for a contrast with the other GLs, which are medium sized and infrequently painful, predominantly nodular, and frequent effect on the subcutaneous tissue and atypia. The information provided here about the clinical and histological characteristics of the different varieties of leiomyomas indicate that there is a need to reconsider the classification of nipple leiomyomas outside the group of GLs. An association between PL and breast carcinoma was detected, which needs to be confirmed in future studies so as to determine if this leiomyoma is a risk marker for breast cancer.

A Rare Case of Broad Ligament Angioleiomyoma

Angioleiomyoma aka Vascular leiomyoma is a benign mesenchymal tumour originating from the smooth muscles and containing thick walled vessels. It is commonly seen in the subcutis of the lower extremities. Although a few cases of uterine angioleiomyoma have been reported, broad ligament angioleiomyoma is extremely rare. Case History: 45 year old multiparous women presented to the OPD with complains of pain and mass in abdomen since 4 months. No history of menstrual irregularities was noted. Ultrasonography revealed a homogenous solid mass in lower abdomen region. The mass was hypodense. A provisional clinical radiological diagnosis of Broad ligament fibroid was made. The patient underwent a surgery for removal of broad ligament mass with bilateral salpingo-oophorectomy and specimen was submitted for histopathological examination. Conclusion: Angioleiomyoma of broad ligament is an extremely rare diagnosis and should be diagnosed correctly with the help of IHC markers.It must be kept in mind as a differential diagnosis of abdominal mass associated with pain to be able to arrive at a correct diagnosis. Keywords: Angioleiomyoma, broad ligament, vascular leiomyoma, mesenchymal tumour, broad ligament fibroid.