Intracranial angioleiomyoma: a case series of seven patients and review of the literature.

PURPOSE: Angioleiomyoma, predominantly arising from the extremities, is a benign soft tissue tumor. Reports on its intracranial location are rare. We assessed clinical, radiological, and pathological features of intracranial angioleiomyoma (iALM) treated at our neurosurgical institution. METHODS: We consecutively enrolled all patients with neuropathologically confirmed iALM treated at a single neurosurgical institution between 2013 and 2021. Clinical and imaging data were collected, and histological tissue sections were analyzed. A review of the literature on iALM was conducted. RESULTS: Seven patients with iALM (four female) with a median age of 45 years (range: 32-76 years) were identified. In three cases, the lesion was found incidentally. In magnetic resonance imaging (MRI), all tumors were hypo- to isointense on T1-weighted, hyperintense on T2-weighted sequences, and gadolinium-enhancing. A strong FLAIR signal was seen in six patients. Surgery consisted of gross total resection in all cases without perioperative complications. Neuropathological staining was positive for smooth muscle actin (SMA) in all lesions. Mature smooth muscle cells arranged around blood vessels were typically observed. The Ki-67 index was ≤ 3%. The patients were discharged after a median of 6 days (range: 4-9 days). During a median follow-up time of 14 months (range: 4-41 months), no tumor recurrence occurred. In the current literature, 42 additional cases of iALM were identified. CONCLUSION: Intracranial angioleiomyoma is a benign soft tissue tumor treated by gross total resection. Tumor morphology and positive staining for SMA lead to the neuropathological diagnosis.

Clinical characteristics and treatment outcomes of angioleiomyoma of the female genital tract: a retrospective cohort study.

BACKGROUND: Angioleiomyoma, a benign tumour composed of smooth muscle cells and thick-walled vessels, is expected to be very rare in the female genital tract. This study aimed to describe the clinicopathological features and treatment outcomes of angioleiomyoma in the female genital tract. METHODS: We retrospectively reviewed 89 women with angioleiomyoma in the genital tract who were treated at Third Xiangya Hospital of Central South University between July 2008 and October 2023. Symptom remission rate was the primary outcome of the study. RESULTS: Angioleiomyomas accounted for 0.6% of leiomyomas of the female genital tract. The average age of the 89 women was 41.8 ± 8.7 years. Seventy women (78.7%) had a history of uterine surgery, of whom two patients had removed uterine angioleiomyoma by laparoscopic myomectomy. The angioleiomyomas of 61 (68.5%) women were located in the uterine corpus, 17 (19.1%) in the broad ligament, 10 (11.2%) in the cervix and only 1 (1.1%) in the vagina. Abnormal uterine bleeding was the main clinical manifestation of angioleiomyomas located in the uterine corpus or cervix, whereas the main clinical manifestation of angioleiomyomas in the broad ligaments was pelvic mass. Of the 89 women, 59 underwent surgery to preserve the uterus, and 30 underwent total hysterectomy or subtotal hysterectomy. The intraoperative blood loss was more than 500 ml (700-4,500 ml) in six women. The symptom remission rate was 100% after surgery. Among the 59 women with preserved uterus, 8 showed multiple uterine leiomyomas during follow-up, but it was difficult to determine whether they were angioleiomyomas. Angioleiomyomas recurred in one women who underwent total hysterectomy. CONCLUSION: Angioleiomyoma is rare in the female reproductive tract, and patients may present with diverse symptoms, which are related to the location of the tumour. Hysterectomy and myomectomy are both effective treatment methods, but the risk of intraoperative bleeding should be recognised for multiple lesions and those with large diameters. Relapse may occur in some patients.

Epidural angioleiomyoma: an extraordinary cause of compressive myelopathy-MRI findings with histopathological correlation.

BACKGROUND: Angioleiomyomas are benign mesenchymal tumors usually located in the limbs, with anecdotal reports in the spine. We present an atypical case of an epidural spine angioleiomyoma presenting with compressive myelopathy symptoms. The diagnosis was suggested based on MRI findings, and subsequently confirmed histopathologically. RESULTS: This is the first known occurrence of pure spinal epidural angioleiomyoma as a source of compressive myelopathy. The imaging presentation, especially the 'dark reticular sign' on MRI, was crucial in suggesting the diagnosis despite the atypical location CONCLUSION: This report serves to raise awareness among clinicians and radiologists about including angioleiomyoma in differential diagnoses for spinal epidural lesions with indicative MRI features. The favorable outcome after surgical intervention underscores the necessity of swift and accurate diagnosis followed by appropriate treatment for such uncommon spinal tumors.

PDGFRB and NOTCH3 Mutations are Detectable in a Wider Range of Pericytic Tumors, Including Myopericytomas, Angioleiomyomas, Glomus Tumors, and Their Combined Tumors.

Pericytic tumors are subclassified as myopericytomas, myofibromas, angioleiomyomas, and glomus tumors according to the current World Health Organization classification. These pericytic tumors form a continuous morphologic spectrum, including those with combined morphology. However, to our knowledge, no widely accepted criteria for classifying tumors with combined morphology are available. Recent studies have identified platelet-derived growth factor receptor-beta (PDGFRB) gene mutations in a subset of myofibromas, myopericytomas, and myopericytomatoses but not in angioleiomyomas. NOTCH receptor 3 (NOTCH3) mutations have been reported in a subset of infantile myofibromatosis. To assess their potential role in classifying pericytic tumors, we investigated PDGFRB and NOTCH3 mutations in 41 pericytic tumors of variable morphology, including some combined forms. Our results show these mutations to be present in a variety of pericytic tumors, such as myopericytomas (PDGFRB, 3/11; NOTCH3, 4/11), myopericytomatoses (1/2; 1/2), myofibromas (3/6; 0/6), angioleiomyomas (2/13; 3/13), and glomus tumors (5/9; 1/9). Point mutations were identified in 3 tumors in PDGFRB exon 12 (Y562C, S574F, and G576S), 12 tumors in PDGFRB exon 14 (M655I, H657L, and N666K), and 9 tumors in NOTCH3 exon 25 (A1480S/T, D1481N, G1482S, T1490A, E1491K, G1494S, and V1512A). All PDGFRB mutations and NOTCH3 G1482S, T1490A, and G1494S mutations were classified as "deleterious/damaging" by ≥4 of 6 pathogenicity prediction tools in silico. Five-mutation-positive tumors, including 1 myopericytoma-angioleiomyoma, 2 myopericytomatoses-myofibroma, 1 myofibroma-myopericytoma and 1 angioleiomyoma-myopericytoma, were of combined morphology. Therefore, we found PDGFRB and NOTCH3 mutations to be detectable in a much wider variety of pericytic tumors than previously reported and confirmed myopericytomas, myofibromas, angioleiomyomas, and glomus tumors as members harboring PDGFRB or NOTCH3 mutations. Our results thus suggest that PDGFRB or NOTCH3 mutations are not useful for subclassifying members of the pericytic tumor family.

Uterine angioleiomyoma with disseminated intravascular coagulation: a case report.

BACKGROUND: Uterine angioleiomyoma is benign tumor that composed of smooth muscle cells and thick-walled vessels. It is a very rare condition reported to present as lower abdominal mass, accompanied by dysmenorrhea and hypermenorrhea. However, its clinical presentation is not known. CASE PRESENTATION: We report the case of a 44-year-old Japanese woman who developed severe anemia with disseminated intravascular coagulation without obvious external bleeding. The patient had a huge abdominal mass of over 20 cm in size, which was thought to be a uterine tumor. She received daily blood transfusions and her condition improved rapidly after she underwent hysterectomy. Pathological examination of the tumor revealed spindle-shaped cells with little atypia and mitosis, and numerous large vessels with smooth muscle and thrombus in the vessels. CONCLUSIONS: Uterine angioleiomyoma was identified as the cause of the coagulation abnormality. CCND2 and AR gene amplification was detected in the tumor. Uterine tumors that present with coagulopathy despite a clinical course suggestive of benign disease should undergo differential diagnosis for uterine angioleiomyoma.

Primary angioleiomyoma of right frontal cranial base with intracranial and extracranial communication.

INTRODUCTION: Primary intracranial angioleiomyoma (ALM) is quite rare and ALM of the adolescent is even rarer. To date, only three cases of adolescents have been reported. MATERIAL AND METHODS: We carefully introduced a new location of intracranial ALM in an adolescent. The clinical, pathological and imaging features of intracranial ALM were described in detail and published literature was reviewed. RESULTS: To our best knowledge, we presented the fourth primary intracranial ALM of adolescent and the first ALM of the right frontal cranial base with intracranial and extracranial communication. We not only summarize the generalities of ALM but also illustrate the difference between adult and adolescent ALM in the aspects of gender and age predominance, etiology, common location and pathologic subtype. CONCLUSIONS: We reported the first ALM of the right frontal cranial base with intracranial and extracranial communication of an adolescent with a good prognosis. We also summarize the generalities of ALM and illustrate the difference between adult and adolescent ALM. Future investigation of control study with large patient cohorts is needed for both adult and adolescent ALM to compare the difference between them.

Angioleiomyoma of the Parotid Gland Fed by the Superficial Temporal Artery.

Angioleiomyoma in the head and neck area is rarely observed. This report introduces 2 cases of angioleiomyoma discovered in the parotid gland, particularly with the superficial temporal artery (STA) as the feeding vessel. Each patient was evaluated preoperatively by clinical manifestation and computed tomography scan, but the difficulty was encountered due to nonspecific radiologic features of angioleiomyoa. Surgical intervention was performed for precise diagnosis and treatment. The tumors were fed from the STA, with the diagnosis of venous type parotid angioleiomyoma. To our knowledge, there have been only one previous report of angioleiomyoma of the parotid gland fed from STA in the literature. Once parotid tumor is discovered, although rare, the possibility of angioleiomyoma should be considered, and the authors recommend prompt surgical excision for accurate diagnosis and treatment.

Current Concepts of Foot and Ankle Angioleiomyoma.

Angioleiomyoma is a benign tumor, which arises from the smooth muscle. It comprises approximately 4.4% of all benign soft tissues' neoplasms and they are commonly located at the lower extremities. They are most frequently found in middle-aged women. Angioleiomyoma is usually presented as a painful solitary lesion in the subcutaneous tissue. Due to the lack of evidence in the literature, the aim of this current concepts review was to provide foot and ankle surgeons the most updated and useful information for diagnosis and management of foot or ankle's angioleiomyoma. The possible diagnosis of angioleiomyoma is rarely thought of before surgery. X-ray, US, MRI, aspiration, scintigraphy, CT and EMG make part of the diagnostic tools available and angioleiomyoma's main characteristics in each of the exams are detailed. Angioleiomyoma cannot be neglected as consequence of delay or mistreatment increases morbidity and the potential risk to malignant transformation.

Incidental superficial soft tissue epithelioid angioleiomyoma.

Epithelioid angioleiomyoma (EALM) is rare in the skin and subcutaneous tissues. To the best of our knowledge, only two previous cases of this tumor have been reported. We document here the case of an 83-year-old woman who underwent complete removal of a squamous cell carcinoma of the retromolar trigone and lymph node dissection of the neck. An incidental EALM was observed in the adipose tissue. The tumor formed a unilocular, poorly demarcated neoplasm measuring 0.3 cm, and showed cavernous angiomatous spaces with villiform growth of large epithelioid cells arranged in clusters. Besides the epithelioid cells of muscular origin, bundles of well-differentiated smooth muscle cells were observed. Epithelioid cells accounted for 70% of the total. The neoplasm originated in the wall of a medium-sized vein. Epithelioid and spindle cells were positive for alpha-smooth muscle actin, calponin, h-caldesmon, and muscle-specific actin. The endothelial cells lining the vascular spaces showed intense and diffuse positivity for CD31 and ERG. The main differential diagnosis includes metastatic carcinoma, melanoma, perivascular epithelioid cell neoplasm, myopericytoma, glomangiomyoma, epithelioid glomus tumor, and epithelioid leiomyosarcoma. This report expands the morphological spectrum of the EALM. Awareness of this uncommon morphologic variant of angioleiomyoma and the use of adequate techniques can avoid misdiagnosis.

Angioleiomyoma of the extremities: correlation of magnetic resonance imaging with histopathological findings in 25 cases.

OBJECTIVE: To identify the characteristic magnetic resonance imaging (MRI) findings in angioleiomyoma and to clarify its relationship with histopathological findings. MATERIALS AND METHODS: We retrospectively analyzed the MRI findings and pathological subtypes in 25 patients with subcutaneous angioleiomyoma of the extremities. Based on the previous reports, MRI findings that could be characteristic of angioleiomyoma were extracted. According to the World Health Organization classification, all cases were classified into three pathological subtypes: solid, venous, and cavernous. The relationship between MRI findings and pathological subtypes was analyzed. RESULTS: The pathological subtypes were solid (n = 10), venous (n = 11), and cavernous (n = 4). The following MRI findings were observed: (a) hypo- or iso-intense linear and/or branching structures on a T2-weighted image (positive total/solid/venous/cavernous: 19/5/10/4, respectively), which we defined as "dark reticular sign"; (b) peripheral hypointense rim on a T2-weighted image (positive total/solid/venous/cavernous: 19/7/8/4, respectively); and (c) presence of any adjacent vascular structures (positive total/solid/venous/cavernous: 6/3/3/0, respectively). Chi-square test showed a significant relationship between dark reticular sign and pathological subtypes (p = 0.0426). The dark reticular sign was found more frequently in the venous and cavernous types than in the solid type. The other MRI findings did not reveal a significant relationship between pathological subtypes. CONCLUSION: We present the largest case series exploring MRI findings in angioleiomyoma. The dark reticular sign was a characteristic MRI finding of angioleiomyoma and was seen in most of the venous and cavernous types, which may facilitate preoperative diagnosis.

Angioleiomyoma of the nasolacrimal duct: case report and literature review.

Angioleiomyomas are benign tumors composed of smooth muscle and vascular endothelium. While infrequent in overall prevalence, they are exceptionally rare in the head and neck. Herein, we describe the case of a 65-year-old female who was found to have an angioleiomyoma of the right nasolacrimal duct. Endoscopic excision of the lesion along with medial maxillectomy and dacryocystorhinostomy was performed without complication. The current report is one of the few reported cases of angioleiomyoma of the lacrimal drainage system.

Inclusion bodies are not uncommon in angioleiomyoma.

BACKGROUND: Leiomyomas with eosinophilic intracytoplasmic inclusion bodies have been described in the urinary bladder, brain, gastrointestinal tract, uterus, and oral cavity but not in the skin. Prompted by our recent experience with a case of cutaneous angioleiomyoma with many inclusion bodies, we hypothesized that similar cases might have been previously overlooked. METHODS: We retrospectively reviewed 30 cases of angioleiomyoma and 10 cases of piloleiomyoma focusing on inclusion bodies. RESULTS: More than 18 inclusion bodies per 250 µm squared were detected in five cases of angioleiomyoma, fewer than 11 bodies in 20 cases, and none in five cases. For the case with numerous inclusion bodies throughout the specimen, special staining was needed to make a diagnosis. No inclusion bodies were found in the piloleiomyomas. CONCLUSION: Inclusion bodies are relatively common in angioleiomyomas and can occasionally be numerous. They may serve as a point of distinction from piloleiomyomas. Because the presence of multiple eosinophilic intracytoplasmic inclusions can result in a rhabdoid appearance and make diagnosis challenging, we should be aware of this feature in angioleiomyomas.

Acral Calcified Vascular Leiomyoma: Report of 3 Cases and Literature Review.

ABSTRACT: Angioleiomyomas are benign neoplasms, which usually present as solitary, slow-growing nodules on the skin of lower extremities, but acral location on the hands or feet is unusual. Yet, microscopically, they may show many histopathological variants, focal calcification is uncommon. Extensive calcification masquerading the real nature of the tumor has been rarely reported, the term acral calcified leiomyoma having been proposed for this entity. This change is more often in distal locations and has been interpreted as degenerative in nature, probably related to repetitive minor trauma. We report 3 examples of this unusual condition on the feet of 2 male and one female subjects (aged, 68, 69, and 80 years) and make a review of the 31 cases available in the literature. Two of our cases are associated with highly uncommon features, such as transepidermal calcium elimination and concomitant calcaneal spur.

A rare case of orbital angioleiomyoma.

A 65-year old woman presented with 3-year history of painless, gradual swelling of the right upper eyelid associated with proptosis. Computed tomography (CT) and magnetic resonance imaging (MRI) of the orbit showed a well circumscribed soft tissue mass in the supero-lateral orbit. An excision biopsy of the lesion was performed via lateral orbitotomy. Histopathology examination and immunochemistry staining confirmed the diagnosis of cavernous angioleiomyoma. The tumour was excised completely. Orbital angioleiomyoma is a rare benign tumour and the lesion can cause visual morbidity, particularly when intraconal. Despite sophisticated imaging modalities, histopathological analysis is essential for diagnosis. Angioleiomyoma should be included in the differential diagnosis of well-defined orbital lesions. Complete surgical excision carries a low risk of recurrence.

A rare case of congenital piloleiomyoma of the eyelid.

Cutaneous leiomyoma is an infrequently occurring benign smooth muscle neoplasm of skin. Piloleiomyoma, angioleiomyoma, and genital leiomyoma are the three forms of the cutaneous leiomyoma. Piloleiomyoma arises from arrector pili muscle and is commonly seen in the adult population. Congenital piloleiomyoma is extremely rare and has never been reported to arise from the eyelid. We report a case of a neonate presenting with upper eyelid mass lesion since birth causing mechanical ptosis. Incisional biopsy followed by histopathology and immunohistochemistry established the diagnosis of piloleiomyoma. There was associated cryptorchidism in our case, a systemic association that has never been reported. Considering the benign nature of the lesion the child was kept under regular follow up without attempting any further removal.

Computed tomography evaluation of extensive intravenous angioleiomyoma: a case report.

BACKGROUND: Uterine angioleiomyoma is a rare variant of leiomyoma, and the main therapy is complete surgery. This study introduces the benefit of three-dimensional computed tomography reconstruction for preoperative preparation. CASE PRESENTATION: A 50-year-old woman presented because of chest distress after activity, with worsening symptoms. After examination, the final diagnosis was uterine angioleiomyoma. The tumour originated in the uterus; grew into the right iliac vein; coursed along the iliac vein, inferior vena cava, and right atrium; and finally invaded the right ventricle. To best complete the surgery, a multidisciplinary surgery was selected. Before the surgery, a three-dimensional computed tomography reconstruction model was created to assess the tumour status, and this model enabled the surgery to be completed successfully. CONCLUSION: Three-dimensional computed tomography reconstruction is of great significance for the preoperative diagnosis of uterine angioleiomyoma and the formulation of surgical treatment plans. Based on its vivid images, surgeons can perform operations more effectively and safely.

Rare giant primary intracranial angioleiomyoma in lateral ventricle: a case report and the literature review.

Background: Angioleiomyomas are benign tumors and can occur in subcutaneous tissues all over the body, and lower extremities are more common. Primary intracranial angioleiomyomas are rare. We present a case of intracranial angioleiomyoma and review the literature.Case description: A 35-year-old Chinese women presented with one-year history of the left leg claudication. MRI revealed a 6.3 × 7.4 × 5.4 cm lesion located in the lateral ventricle, which, to our knowledge is the first lateral ventricle ALM reported. The tumor was resected. The pathological results were consistent with angioleiomyoma. Hemiplegia of left limb was found during post-operative period and no recurrence was found during five month of follow-up.Conclusion: ALM is a rare intracranial tumor but can occur.

Angioleiomyoma of the lumbo-sacral region with unusual keloid-like collagen fibers.

Angioleiomyoma is a benign soft tissue tumor which usually occurs in superficial or deep soft tissues. Only rarely does this tumor occur at unusual sites, including retroperitoneum. We present a rare case of lumbo-sacral angioleiomyoma in a 54-year-old man. Apart from this unusual site, the most striking morphological feature was the presence of numerous keloid-like collagen fibers interspersed among the fascicles of the neoplastic cells. Radiological, morphological and immunohistochemical features are presented, and differential diagnosis with its potential morphological mimickers is discussed.

Nipple leiomyoma: A rare neoplasm with a broad spectrum of histologic appearances.

Cutaneous leiomyomas are rare benign smooth-muscle tumors. These lesions are distinguished based on their cell of origin and are subclassified as pilar leiomyoma, angioleiomyoma, and genital-type leiomyoma. Nipple leiomyoma is the least common genital-type leiomyoma, arising from the dartoic muscle cell of the nipple. Histologic examination of the lesion is necessary for definitive diagnosis, and these uncommon tumors can pose a diagnostic challenge. We describe herein a series of six nipple leiomyomas with a spectrum of histologic appearances.

Dermoscopy of cutaneous smooth muscle neoplasms: a morphological study of 136 cases.

BACKGROUND: A variety of cutaneous smooth muscle neoplasms may arise in the skin and are frequently unrecognized by clinicians. There is sparse data relating to the dermoscopy of piloleiomyomas (PL), and nothing has been published about the dermoscopy of angioleiomyomas (AL) and leiomyosarcomas (LS). OBJECTIVES: To evaluate the morphological findings of a large series of cutaneous PL, AL and LS under dermoscopic observation, comparing these findings among them. METHODS: Digital dermoscopic images of 136 histopathologically confirmed cases of cutaneous smooth muscle neoplasms (114 PL, 13 AL and 9 LS) collected from 10 Hospitals in Spain, Austria and Italy were evaluated for the presence of dermoscopic structures and patterns. RESULTS: The pattern composed of a symmetric, total delicate pigment network with the variable presence of multiple hypopigmented areas in a painful lesion is the most common dermoscopic pattern associated with PL. This pattern was found in 69.3% of PL and in no cases of AL and LS. The most common and characteristic pattern associated with AL was the one composed of symmetric pink-reddish tumour with vessels, white structures and the absence of ulceration, which was found in 46.2% of AL, but also in 3.5% of PL, and in 22.2% of LS. Finally, the most common pattern associated with LS was the one composed of an asymmetric, multilobulated tumour with linear-irregular or polymorphic-atypical vessels and white structures, which was found in 44.4% of cases, but also in 0.9% of PL and in 15.4% of AL. CONCLUSION: Dermoscopy is helpful in improving the diagnostic accuracy of PL. The dermoscopic patterns associated with AL and LS were more variable and less specific.