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Lipomas of the palatine tonsil are rare benign neoplasms in clinical practice. We present a case of palatine tonsillar fibroangiolipoma in a 50-year-old Chinese male with a history of multiple lipomas on the back and extremities. It was diagnosed based on histological examination and integrated analysis. Good wound healing and no evidence of recurrence were noted within 6 months follow-up after tonsillectomy. This article also puts a spotlight on the differential diagnosis of benign tonsillar tumors and reviewed recent relevant literature., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Spinal epidural angiolipomas are rare, benign, mesenchymal tumors. It remains unclear whether spinal epidural angiolipomas are genuinely rare or merely underreported. Herein, we assessed the demographic and clinical characteristics of patients with spinal epidural angiolipoma. We collected data from patients with spinal epidural angiolipoma from three sources. First, we retrospectively analyzed data from patients diagnosed with spinal epidural angiolipoma in our hospital between January 1, 2014, and December 31, 2023. Second, we performed a literature review of studies retrieved from PubMed. Third, we retrieved detailed data of patients with spinal angiolipoma from the Surveillance, Epidemiology, and End Results (SEER) database. We conducted a descriptive analysis to investigate the demographic and clinical characteristics of patients with spinal epidural angiolipoma. At our institution, three patients were diagnosed with spinal epidural angiolipoma. Additionally, we identified 116 patients from the literature review and 15 patients from the SEER database. We reviewed the treatment history and imaging features of the three patients from our institution. The descriptive analysis of the data collected from the literature review was consistent with previous reports. For example, 63.0% of lesions were located at the thoracic level. 31.9% of these lesions involved two vertebral bodies, while 75.6% involved 2-4 vertebral bodies. The most common symptoms experienced by patients were back pain, paraparesis, and numbness in the legs. Surgery was the primary treatment option for most patients, and complete tumor resection was achieved in the majority of patients. The male:female ratio was 1:1.4, the median age at diagnosis for the patients from the literature was 49 years old, and the median follow-up was 24 months. Notably, most of the reports came from Asia and there were few reports from Africa. The findings from the SEER database indicated a male:female ratio of 2:1. The peak incidence, which is typically reported in the fifth decade of life, was not observed. We presented three cases of spinal epidural angiolipoma and supplemented our findings with a literature review and population-based analysis according to the SEER database for the United States population. We believe that our research will enhance clinicians' comprehension of this uncommon tumor., (© 2024. The Author(s).)

Background: Angiolipomas have been well described in patients with HIV exposed to protease inhibitors with possible resolution after switching to non-nucleoside reverse transcriptase inhibitor-based regimens. Resolution of symptoms have occurred with switches to non-nucleoside reverse transcriptase inhibitor (NNRTI)-based regimens; however, little is known regarding the development of angiolipomas when switching from NNRTI- to modern, integrase strand transfer inhibitor-based regimens. We describe a patient who underwent switch therapy from tenofovir disoproxil fumarate/emtricitabine/efavirenz (TDF/FTC/EFV) to tenofovir alafenamide/FTC/bictegravir (TAF/FTC/BIC) who later developed angiolipomas., Case Presentation: A 55-year-old male had been on TDF/FTC/EFV for 8 years before switching to TAF/FTC/BIC. Nineteen months after antiretroviral switch, the patient presented with multiple lesions in the upper extremities and abdomen. Diagnostic biopsies revealed non-encapsulated angiolipomas and HHV-8 and non-alcoholic fatty liver disease was ruled out. New lesions continued to appear 29 months after ART switch, after which now lesions appeared and prior lesions remained stable with no increase in size noted. No surgical intervention or change in antiretroviral therapy was needed., Conclusions: Angiogenesis may have been suppressed with TDF/FTC/EFV treatment, however when switched to TAF/FTC/BIC, promoted the growth of angiolipomas. Clinicians should be aware of the impact of switching to modern ART therapies resulting in possible adipogenesis., (© 2024. The Author(s).)

Esophageal angiolipoma is a rare disease with unspecific clinical manifestations.This paper reported a case of esophageal angiolipoma confirmed by upper gastrointestinal endoscopy and summarized the clinical manifestations,endoscopic and pathological features,treatment and prognosis of the patients by reviewing the relevant literature,aiming to provide references for clinical diagnosis and treatment of this disease in the future.

Background:  Spinal angiolipomas (SAs) are rare, benign tumors, representing 0.0004 to 1.2% of angiolipomas, usually located at the extradural and posterior thoracic level, with multimetameric extension., Methods:  A systematic literature review was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. The authors searched on PubMed and Scopus databases for published articles with the Mesh term "spinal angiolipoma" and pertinent associations. Language restriction to English papers was applied. The authors also reported three emblematic cases of patients who underwent surgical resection of spindle-shaped (type IA) and dumbbell-shaped (type II) SAs between 2014 and 2020., Results:  Of the 256 retrieved articles, 33 were included in the meta-analysis. These 33 studies, together with our 3 reported cases, included a total of 60 patients, 36 females (60%) and 24 males (40%), with a mean age of 53.12 ± 12.82 years (range: 12-77 years).T5 was the most represented level (22 patients). Usually, the localization of SA was extradural, with 53 patients suffering from spindle-shaped type IA SA (88.3%) and 7 patients from dumbbell-shaped type II SA (11.6%). Almost all patients underwent laminectomy (78.3%) and presented a full recovery of motor deficits (85%). Gross total removal (GTR) was performed in 93.3% of patients. The mean follow-up was of 22.71 ± 21.45 months (range: 2-80 months). There was no documented recurrence at follow-up magnetic resonance imaging in any of the patients., Conclusions:  SAs are rare, benign tumors with a great vascular component that presents a favorable outcome. GTR is the gold standard treatment and usually an adjuvant therapy is not required. Even infiltrative lesions, which are more complex, can be treated successfully with a good prognosis. Dumbbell-shaped SAs must be differentiated from schwannomas and meningiomas, and require different surgical techniques, given the profuse bleeding associated with the attempt of debulking. En block resection is the key to treat these common benign tumors with acceptable blood loss., Competing Interests: None declared., (Thieme. All rights reserved.)

Background: Angiolipoma (AL) is considered as a lipoma variant that is characterized by the combination of mature adipocytes and capillary blood vessels diffusely distributed within the tumor. With the exception of recurrent PRKD2 mutations of uncertain pathogenetic significance, the genetic abnormalities of ALs are unknown, in the absence of any of the specific chromosomal aberrations described in other lipoma variants., Methods: Formalin-fixed and paraffin-embedded blocks of 13 conventional ALs and 5 cellular ALs from 17 individuals were retrieved and analyzed for mutations in exons 9 and 20 of PIK3CA by polymerase chain reaction and Sanger sequencing., Results: Activating PIK3CA mutations were identified in 14 tumors (78%). All PIK3CA-mutated samples carried the same exon 9 mutation, c.1634A>C (p.E545A). No mutation was detected in exon 20 of PIK3CA. No significant difference between PIK3CA-mutated and wild-type samples appeared to exist based on age, gender, and location of the tumor. All 5 cellular ALs carried the p.E545A PIK3CA mutation., Conclusion: The high frequency of the p.E545A PIK3CA mutation in both conventional and cellular ALs suggests that activation of the PI3K/AKT pathway plays a key role in AL pathogenesis and reinforces the concept that cellular AL should be regarded as a variant of AL., (© 2020 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Mesenchymal lesions of the gastrointestinal tract are generally uncommon compared with epithelial derived entities. Angiolipofibroma describes a rare gastrointestinal tract mesenchymal lesion composed of varied amounts of adipose tissue, fibrous tissue, along with admixed blood vessels. Descriptions of this entity are limited to few case reports describing a total of 5 lesions. Angiolipofibroma represents a benign entity that may uncommonly present as a mass lesion concerning for malignancy. The etiology is unclear; however, these may represent a reactive or hamartomatous process. In this article, we sought to further describe this entity and present an additional 11 cases.

Purpose: To investigate associations between clear-cell renal cell carcinoma mRNA-based subtyping and CT features., Materials and Methods: The CT data from 177 patients generated with The Cancer Imaging Archive were reviewed. The correlation was analyzed using chi-square test and univariate regression analysis., Results: Identified were 124 (53.2%) m1, 67 (28.8%) m2, 17 (7.3%) m3, and 14 (8.7%) m4 subtypes. m1-subtype rates were significantly higher in well-defined margin lesions (p = 0.041). m3-subtype rates were significantly higher in ill-defined margin lesions (p = 0.012), in collecting system invasion lesions (p = 0.028) and collecting system invasion lesions (p = 0.026).On univariate logistic regression analysis, tumor margin (well-defined margin vs ill-defined margin, OR: 2.104; p = 0.041; 95% CI: 1.024-4.322) was associated with m1-subtype. Tumor margin (well-defined margin vs ill-defined margin, OR: 2.104; p = 0.012; 95% CI: 0.212-0.834) and collecting system invasion (yes vs no, OR: 0.421; p = 0.028; 95% CI: 0.212-0.834) and renal vein invasion (yes vs no, OR: 2.164; p = 0.026; 95% CI: 1.090-4.294) were associated with m3-subtype. There was no significant difference between mRNA-based subtyping (m2 vs other; m4 vs other) and the CT features., Conclusions: This preliminary radiogenomics analysis of clear-cell renal cell carcinoma revealed associations between CT features and mRNA-based subtyping which warrant further investigation and validation., (Copyright © 2018 The Association of University Radiologists. Published by Elsevier Inc. All rights reserved.)

Spinal angiolipomas are benign tumors predominantly found in the epidural space. Infiltrating spinal angiolipoma is an extremely rare type, which tends to invade the surrounding tissue and may mimic an aggressive tumor. Herein, we reported the case of a 64-year-old woman with a thoracic angiolipoma involving the T9 vertebral body and its appendix. The lesion showed long T1 and T2 signal intensity on MRI and slightly increased FDG uptake on PET/CT. After surgery, the pathologic examination demonstrated features consistent with angiolipoma, and the patient was free of symptoms.

A 63-year-old woman presented to the emergency unit with a huge (40 × 35 × 10 cm) oval-shaped pedunculated growth in the back with superficial ulceration in its lower aspect, associated with signs of sepsis. After adequate resuscitation, the patient was transferred to the operation theatre where the tumour was completely excised and the resultant wound was closed in primary manner using polypropylene sutures with suction drains inserted in the wound bed. The patient was discharged home after two days and was followed-up for two months. No wound complications were recorded on follow-up. Angiolipoma is a benign variant of lipoma that, despite what has been reported in the literature, may affect any age and any region of the body. Complications of angiolipoma may include ulceration, sepsis, gangrene and possible systemic inflammatory response syndrome which may pose a threat to the patient's life, hence should be managed promptly after adequate patient preparation.

Introduction: We present our experience with the diagnosis and treatment of spinal angiolipomas (SAL)., Methods: We used our database of surgical patients to gather epidemiological, clinical, diagnostic, treatment, and follow-up data from confirmed cases of SAL. Patients' functional status was evaluated using the modified Japanese Orthopaedic Association (mJOA) score for thoracic myelopathy., Results: We included 7 patients with ages ranging between 42 and 63 years; 4 were women. Lesions in all patients were located in the thoracic epidural space; in 2 cases, lesions extended outside the spinal canal. The most frequent form of presentation was pain combined with progressive myelopathy; patients scored 5 to 9 points on the mJOA scale. Radiological findings varied; this variability clearly depended on the proportion of tumour components (cellular variant in one case, predominantly angiomatous in another, and myxoid in yet another case). Four cases displayed radiological signs of bone involvement. Surgery resulted in excellent mJOA scores except in one case (mJOA score of 10); no recurrences were detected during follow-up (periods ranging from 1 to 5 years)., Conclusions: Extradural SAL can present with highly variable radiological and clinical features. Surgery must be tailored to each individual situation according to tumour extension; in some cases, it may include spinal reconstruction. Surgery results in excellent outcomes regardless of preoperative functional status and it is associated with low recurrence rates, even in case of incomplete resection., (Copyright © 2016 Sociedad Española de Neurología. Publicado por Elsevier España, S.L.U. All rights reserved.)

Spinal angiolipomas (SALs) are rare extradural tumors frequently located at the level of the thoracic spine and they are associated with spinal cord compromise that might result in severe myelopathy. While the first macroscopic description dates 1890, histologically these tumors where not described as angiolipomas until 1986 by Haddad et al. Occurrence in pregnancy is even more infrequent. Since their first macroscopic description, spinal angiolipomas were reported anecdotally in pregnant women. We present a case of spinal angiolipoma in pregnancy with confirmed histologic diagnosis. In the present paper, we reviewed the literature regarding spinal angiolipomas in order to characterize their clinical manifestation, natural history, radiologic and histologic appearance. We add also a further case of spinal angiolipoma in a pregnant woman. Finally, we provide suggestions for the management of such rare tumors in pregnancy., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Angiolipoma, a subtype of lipoma, is a benign adypocytic soft tissue tumor composed of mature adipose tissue and small vascular proliferations. This entity makes up 5-17% of all lipomas. The diagnosis is made by clinical and pathological examination, ultrasonography, and/or magnetic resonance imaging (MRI). It is generally an encapsulated tumor (noninfiltrative), but rarely has an infiltrative form. Angiolipoma mostly occurs on the trunk and extremities with male predominance. The forearm is the most frequent location for angiolipomas. It is very rarely seen on the fingers. Herein, we report a patient with solitary noninfiltrating angiolipoma on a finger. The patient was treated with surgical excision and no recurrence has been noted over one year of observation.

Background: Primary angiosarcoma of the kidney is a rare and aggressive malignant tumor presenting with a recognizable vascular differentiation. Its prognosis is fatal and the pathogenesis remains unclear. Renal angiomyolipoma is a relatively infrequent renal cortical neoplasm and is composed of variable proportions of adipose tissue, spindle cells, epithelioid smooth muscle cells and abnormal thick-walled blood vessels., Case Presentation: Here, we reported a case in which a 64-year-old woman presenting with the chief complaint of a progressively enlarged mass in the left abdomen. Abdominal computed tomography confirmed presence of a tumor measuring 18 cm × 11 cm in the left posterior renal fascia. Microscopic examination and immunohistochemical staining confirmed co-existence of angiomyolipoma and angiosarcoma. Furthermore, the two components interspersed with each other and there were transitional zones between them., Conclusions: In this case, we described for the first time a primary renal angiosarcoma possibly arising in a pre-existing angiomyolipoma of the kidney.

Angiolipoma is a benign soft tissue tumor with two subtypes: non-infiltrating and infiltrating. Although histologically benign, infiltrating angiolipoma can invade surrounding structures. The foot is a very rare location for angiolipoma, with only four cases reported in the English literature, including one infiltrating type. Here, we report a very rare case of infiltrating angiolipoma of the foot with magnetic resonance imaging (MRI) and ultrasonography (US) findings. A 7-year-old boy presented with a slowly growing foot mass. MRI showed an unencapsulated mass involving the third web space extending to the foot dorsum and sole. The mass was isointense to subcutaneous fat and was mixed with internal T1 low-signal-intensity enhancing areas. On US, we observed a heterogeneously hypoechoic mass with internal vascularity. Imaging and clinical features of angiolipoma and the radiologic differential diagnoses of a fat-containing mass in the pediatric foot are reviewed here. When there is an ill-defined foot mass with a fat component and variable enhancing portions in a child, infiltrating angiolipoma should be included in the differential diagnosis along with other fat-containing tumors.

Background/objective: To present the long-term result and efficacy of robotic partial nephrectomy (RPN) for renal angiomyolipomas (AMLs) with perioperative outcome and renal function preservation., Methods: From September 2006 to October 2014, the database of a single medical center was reviewed and patients who underwent RPN for AMLs were enrolled. The patient demographics, perioperative complications, and postoperative outcomes were analyzed., Results: We identified 23 patients who were treated with RPN for renal AMLs. The average age was 52.7 (± 9.9) years, and 20 (87%) patients were female. The median size of the resected AML was 5.2 [interquartile range (IQR)=3.1-6.8] cm. The median estimated blood loss was 100 (IQR=50-225) mL, and three (13%) patients required blood transfusion. Perioperative complications occurred in six (26%) patients and none of them are higher than Clavien Grade II. The median estimated glomerular filtration rate at 3-month and the latest follow-ups were 103 (IQR=85.5-112) mL/min/1.73m 2 and 104 (IQR=90-112) mL/min/1.73m 2 , respectively, with a median of 89.6% (IQR=84.2-100) and 86.9% (IQR=81.3-97.8) preservation, respectively. The median follow-up period was 40 (IQR=30.5-61.5) months. None of the patients developed complications requiring a second intervention or local recurrence of AML., Conclusion: A long-term follow-up of RPN for renal AMLs revealed good preservation of renal function with a low complication rate. It may be considered as a reliable method to manage renal AMLs., (Copyright © 2016. Published by Elsevier Taiwan.)

Background/aim: Angiolipoma is a rare benign soft tissue tumor composed of mature adipocytes and blood vessels. Genetic information on angiolipomas is scarce. With the single exception of one tumor which carried a t(X;2)(p22;p12), all angiolipomas hitherto investigated cytogenetically had normal karyotypes., Materials and Methods: G-banding chromosome analysis was performed on three short-term cultured angiolipomas. Fluorescence in situ hybridization (FISH) analysis using a commercially available RB1 deletion probe was also done., Results: All three angiolipomas had abnormal karyotypes with loss or structural rearrangement of chromosome 13. The first tumor had the karyotype 46,XY,-6,del(13)(q14),+mar[cp5], the second had 44~45,XY,t(1;10;15)(p21~22;q24;q24),-13[cp5], and the third karyotype was 43,XX,t(13;22;17) (q12;q13; q22~23)[14]. FISH analysis showed heterozygous and homozygous deletion of the RB1 probe in case 2 and 3, respectively. FISH analysis failed in case 1., Conclusion: Chromosome 13 was consistently involved in all three angiolipomas., (Copyright© 2018, International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.)

Breast tumors with lipomatous or liposarcomatous components are infrequently encountered, but can be a source of diagnostic difficulty if the context of the fatty differentiation is not recognized. Among the true adipocytic tumors, lipoma is the most common lipomatous tumor arising in the breast. Several mammary spindle cell tumors may show adipocytic differentiation, including fibroepithelial tumors and myofibroblastoma. Liposarcomatous components most often arise in malignant phyllodes tumors, as opposed to primary liposarcomas of the breast which are believed to be uncommon. This article will review the spectrum fat-containing tumors of the breast with an emphasis on differential diagnosis and insights from recent molecular studies., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Competing Interests: Disclosure The authors report no conflicts of interest in this work.

Angiolipoma is a benign tumor composed of adipose tissue and proliferating blood vessels that is commonly found in the subcutaneous tissue of the trunk and extremities. Gastric angiolipoma is a rare entity, and to the best of our knowledge, only 4 cases have been reported in the English-language literature thus far. These tumors may present as gastrointestinal bleeding and anemia or with obstructive symptoms. Accurate preoperative diagnosis is challenging because of nonspecific clinical symptoms and lack of specific findings on imaging studies. The correct diagnosis is usually made by histopathologic examination. The clinical significance lies in being aware of this rare entity in the stomach and distinguishing it from other benign and malignant gastric neoplasms that may be in the differential diagnosis. We herein discuss the clinical presentation, radiologic and histopathologic features, ancillary studies, differential diagnosis, and treatment and prognosis of this rare entity.

Tumours displaying differentiation towards normal fat constitute the most common subgroup of soft tissue neoplasms. A series of such tumours was investigated by whole-exome sequencing followed by targeted ultra-deep sequencing. Eighty per cent of angiolipomas, but not any other tumour type, displayed mutations in the protein kinase D2 (PRKD2) gene, typically in the part encoding the catalytic domain. The absence of other aberrations at the chromosome or RNA level suggests that PRKD2 mutations are critical for angiolipoma development. Consistently, the mutated PRKD2 alleles were present at low (3-15%) frequencies, indicating that only a subset of the tumour cells is affected. Indeed, by sequencing mature fat cells and other cells separately, the former typically showed the highest mutation frequencies. Thus, we hypothesize that altered PRKD2 signalling in the adipocytic cells drives tumourigenesis and, in agreement with its pivotal role in angiogenesis, induces the vessel formation that is characteristic for angiolipoma. Copyright © 2016 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd., (Copyright © 2016 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.)

Background: Although percutaneous endoscopic technique has been routinely used in the treatment of disk herniation, there are few reports on its application in the management of intraspinal tumors. We present a case report of lumbar epidural angiolipoma that was totally removed by percutaneous endoscopic technique., Case Description: A 63-year-old man presented with a 4-month history of progressively worsening low back pain. No abnormal neurologic finding was noted on physical examination, and magnetic resonance imaging demonstrated a dorsally located L2-3 epidural lesion, suggestive of a noninfiltrating angiolipoma. During the operation, an 8-mm skin incision was made, and a dilator was bluntly inserted toward the interlaminar space, followed by insertion of a working cannula onto the ligamentum flavum and placement of the endoscope. The interlaminar space was enlarged by resection of part of the lower rim of the right L2 vertebral laminae, and the right side of the interlaminar ligamentum flavum was removed to expose the tumor. The tumor was totally removed piecemeal under endoscopic guidance, and pathologic examination confirmed the diagnosis of angiolipoma., Conclusions: This report supports the application of percutaneous endoscopic technique in the surgical resection of noninfiltrating extradural lumbar angiolipoma., (Copyright © 2016 Elsevier Inc. All rights reserved.)

The fusion or amplification of TFE3 has been identified as one of the molecular events underlying tumorigenesis in perivascular epithelioid cell tumors (PEComas). TFE3 rearrangements in PEComas are related to the morphological features of the epithelioid appearance and weaker expression of immunohistochemical muscular markers. This study aimed to clarify whether these genetic alterations are involved in hepatic angiomyolipomas (AMLs), which are a member of the PEComa tumor family. We examined 28 liver specimens (15 biopsies and 13 surgical specimens) of hepatic AMLs obtained from 26 patients. Renal AMLs (n=20), extrahepatorenal PEComas (n=3), lymphangiomyomatosis (n=8), and hepatocellular carcinomas (n=40) were used as a control. A histologic comparison between hepatic and renal AMLs revealed that the epithelioid appearance was more common in hepatic tumors (38% versus 0%, P=.006). In immunohistochemistry, the expression of HMB45 and Melan-A appeared to be more widespread in hepatic AMLs than in renal AMLs, whereas smooth muscle actin and desmin were less broadly expressed in hepatic tumors (all P<.001). TFE3 also appeared to be overexpressed in 6 (21%) of 26 hepatic AMLs and 3 (100%) of 3 PEComas, but in none of the renal AMLs. In fluorescence in situ hybridization, although all PEComas harbored a TFE3 rearrangement or amplification, no genetic alterations were found in any hepatic AMLs. In conclusion, although hepatic AMLs and TFE3-rearranged PEComas share pathological features such as the epithelioid appearance and immunoreactivity to TFE3, TFE3 alterations are less likely to be a major molecular event driving tumorigenesis in hepatic AMLs., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background: Angiolipoma is a rare benign neoplasm composed of mature fatty tissue and multiple small abnormal blood vessels. Infiltrating mediastinal angiolipoma is an extremely rare tumor associated with delayed diagnosis., Case Presentation: A 42-year-old woman was presented with 3-month history of mild chest tightness. Imaging of the chest showed a large mass with fat densities in the middle superior mediastinum. A presumptive diagnosis was a tumor of liposarcoma. The patient was scheduled for a thoracotomy. After the excision, the symptoms were relieved and histological study revealed that the tumor was an angiolipoma. The patient recovered very well and was discharged 7 days after the surgery. After 7 months of follow-up the patient was clinically well and asymptomatic., Conclusions: We described a giant infiltrating mediastinal angiolipoma and its removal, and discussed the tumor characteristics and prognosis. Although extremely rare, infiltrating angiolipoma should be considered in the differential diagnosis of mediastinum lesions. The prognosis after surgical management of our patient is favorable.

Background: Angiogliomas are rare low-grade glial tumors with significant vascular components. These tumors are usually seen in the brain, and spinal cord angiogliomas have not been reported in the literature until now., Case Description: We report the case of a 15-year-old boy with an angioglioma of the medulla and cervicodorsal spine, which was completely excised through a combined suboccipital craniotomy and cervicodorsal laminotomy. The patient experienced excellent clinical recovery after the surgery, and follow-up contrast magnetic resonance imaging showed complete excision of the tumor., Conclusion: The fact that increased vascularity in a glioma does not always indicate a higher grade is confirmed by the unique histology of angiogliomas. These tumors can present with intratumoral bleeding. Awareness of these entities is extremely important. Complete excision can be attempted, and the postoperative prognosis is very good., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background: Previous studies have shown potential benefits of rapamycin or rapalogs for treating people with tuberous sclerosis complex. Although everolimus (a rapalog) is currently approved by the FDA (U.S. Food and Drug Administration) and the EMA (European Medicines Agency) for tuberous sclerosis complex-associated renal angiomyolipoma and subependymal giant cell astrocytoma, applications for other manifestations of tuberous sclerosis complex have not yet been established. A systematic review is necessary to establish the clinical value of rapamycin or rapalogs for various manifestations in tuberous sclerosis complex., Objectives: To determine the effectiveness of rapamycin or rapalogs in people with tuberous sclerosis complex for decreasing tumour size and other manifestations and to assess the safety of rapamycin or rapalogs in relation to their adverse effects., Search Methods: Relevant studies were identified by authors from the Cochrane Central Register of Controlled Trials (CENTRAL), Ovid MEDLINE, and clinicaltrials.gov. Relevant resources were also searched by the authors, such as conference proceedings and abstract books of conferences, from e.g. the Tuberous Sclerosis Complex International Research Conferences, other tuberous sclerosis complex-related conferences and the Human Genome Meeting. We did not restrict the searches by language as long as English translations were available for non-English reports.Date of the last searches: 14 March 2016., Selection Criteria: Randomized or quasi-randomized studies of rapamycin or rapalogs in people with tuberous sclerosis complex., Data Collection and Analysis: Data were independently extracted by two authors using standard acquisition forms. The data collection was verified by one author. The risk of bias of each study was independently assessed by two authors and verified by one author., Main Results: Three placebo-controlled studies with a total of 263 participants (age range 0.8 to 61 years old, 122 males and 141 females, with variable lengths of study duration) were included in the review. We found high-quality evidence except for response to skin lesions which was judged to be low quality due to the risk of attrition bias. Overall, there are 175 participants in the treatment arm (rapamycin or everolimus) and 88 in the placebo arm. Participants all had tuberous sclerosis complex as proven by consensus diagnostic criteria as a minimum. The quality in the description of the study methods was mixed, although we assessed most domains as having a low risk of bias. Blinding of treatment arms was successfully carried out in all of the studies. However, two studies did not report allocation concealment. Two of the included studies were funded by Novartis Pharmaceuticals.Two studies (235 participants) used oral (systemic) administration of everolimus (rapalog). These studies reported response to tumour size in terms of the number of individuals with a reduction in the total volume of tumours to 50% or more relative to baseline. Significantly more participants in the treatment arm (two studies, 162 participants, high quality evidence) achieved a 50% reduction in renal angiomyolipoma size, risk ratio 24.69 (95% confidence interval 3.51 to 173.41) (P = 0.001). For the sub-ependymal giant cell astrocytoma, our analysis of one study (117 participants, high quality evidence) showed significantly more participants in the treatment arm achieved a 50% reduction in tumour size, risk ratio 27.85 (95% confidence interval 1.74 to 444.82) (P = 0.02). The proportion of participants who showed a skin response from the two included studies analysed was significantly increased in the treatment arms, risk ratio 5.78 (95% confidence interval 2.30 to 14.52) (P = 0.0002) (two studies, 224 participants, high quality evidence). In one study (117 participants), the median change of seizure frequency was -2.9 in 24 hours (95% confidence interval -4.0 to -1.0) in the treatment group versus -4.1 in 24 hour (95% confidence interval -10.9 to 5.8) in the placebo group. In one study, one out of 79 participants in the treatment group versus three of 39 in placebo group had increased blood creatinine levels, while the median percentage change of forced expiratory volume at one second in the treatment arm was -1% compared to -4% in the placebo arm. In one study (117 participants, high quality evidence), we found that those participants who received treatment had a similar risk of experiencing adverse events compared to those who did not, risk ratio 1.07 (95% confidence interval 0.96 - 1.20) (P = 0.24). However, as seen from two studies (235 participants, high quality evidence), the treatment itself led to significantly more adverse events resulting in withdrawal, interruption of treatment, or reduction in dose level, risk ratio 3.14 (95% confidence interval 1.82 to 5.42) (P < 0.0001).One study (28 participants) used topical (skin) administration of rapamycin. This study reported response to skin lesions in terms of participants' perception towards their skin appearance following the treatment. There was a tendency of an improvement in the participants' perception of their skin appearance, although not significant, risk ratio 1.81 (95% confidence interval 0.80 to 4.06, low quality evidence) (P = 0.15). This study reported that there were no serious adverse events related to the study product and there was no detectable systemic absorption of the rapamycin during the study period., Authors' Conclusions: We found evidence that oral everolimus significantly increased the proportion of people who achieved a 50% reduction in the size of sub-ependymal giant cell astrocytoma and renal angiomyolipoma. Although we were unable to ascertain the relationship between the reported adverse events and the treatment, participants who received treatment had a similar risk of experiencing adverse events as compared to those who did not receive treatment. Nevertheless, the treatment itself significantly increased the risk of having dose reduction, interruption or withdrawal. This supports ongoing clinical applications of oral everolimus for renal angiomyolipoma and subependymal giant cell astrocytoma. Although oral everolimus showed beneficial effect on skin lesions, topical rapamycin only showed a non-significant tendency of improvement. Efficacy on skin lesions should be further established in future research. The beneficial effects of rapamycin or rapalogs on tuberous sclerosis complex should be further studied on other manifestations of the condition.

Introduction Angiolipoma is a histological variant of lipoma and is the most common neoplasm in the trunk and extremities of young adults. It is extremely rare in elderly people, and its size is ≤4cm. Few data are available for large angiolipomas. Case History An 86-year-old patient was admitted to our surgical department due to a large mass on his left arm, which was resected. The specimen measured 19.5 × 15 × 10.5cm. Histopathological examination revealed a benign non-infiltrating angiolipoma. This is the first report of a giant angiolipoma of the arm reported in an octogenarian patient. Conclusions Giant lipomas of the upper extremities are extremely rare. Resection is associated with cure in most patients, but regular follow-up should be considered.

Aim: To improve the clinical diagnosis and recognition of hepatic epithelioid angiomyolipoma (HEAML)., Methods: Four cases of primary HEAML were confirmed based on the pathology archive system in our hospital from January 2009 to November 2015. The general state, clinical symptoms, imaging manifestations, histological results and immunohistochemistry of these patients were retrospectively reviewed and analyzed. Studies of HEAML published in the last 15 years were collected from PubMed and MEDLINE to summarize the clinical symptoms, imaging characteristics, pathological features and management of HEAML., Results: Four cases of primary HEAML were retrieved from our archives. These included three female patients and one male patient, with a mean age of 41.8 ± 11.5 years (ranging from 31 to 56 years). The mean tumor size was 7.3 ± 5.5 cm (ranging from 3.0 to 15 cm). In the contrast-enhanced imaging, the tumor was obviously enhanced in the arterial phase, but enhanced continuously or exhibited a slow-density masse during the venous and delayed phases. Histologically, the tumors mainly consisted of epithelioid cells that comprised approximately 95% of the total neoplastic mass. Although no metastases occurred in our patients, pathological studies revealed necrosis, mitotic figures and liver invasion in two patients, which indicates aggressive behavior. Immunohistochemical staining revealed that human melanoma black 45 (HMB-45) and Melan-A were positive in 4 cases. We only identified 81 cases with primary HEAML, including our present patients, from 26 articles available from PubMed and MEDLINE. The majority of the papers were published as case reports. Only 5 (5/75, 6%) cases were associated with tuberous sclerosis complex (TSC). More than half (35/66) were discovered incidentally upon physical examination. Approximately 65% (22/34) of the patients were misdiagnosed with HCC or other tumors before surgery. Approximately 10% (8/81) of the patients with HEAML had recurrence or metastasis after surgery, which was a very high and alarming rate., Conclusion: HEAML is a very rare primary hepatic tumor that is often misdiagnosed before surgery. Patients should be followed closely after surgery because of its malignant potential.

Background: Angiofibrolipomas are one of the rarest variants of lipomas. Laryngeal origin of lipomatous tumors is also very rare., Methods: The nature and severity of symptoms depend on the location and the size of the laryngeal tumors. However, pedunculated of a 28-cm long tumor with the point of origin on the left arytenoepiglottic fold reached down to the esophagus and. in spite of slight dysphagia. remained asymptomatic for many years. In this case, a narrowing of the digestive tract was masked by an adaptation to the presence of a slowly growing mass., Results: After surgical removal of the tumor and 18-month follow-up, the patient remains asymptomatic. Nonetheless, taking into consideration a possibility of recurrence or malignance, a postoperative regular 5-year follow-up was recommended., Conclusion: This case report illustrates the successful transoral resection of a polypoid-shaped angiofibrolipoma of the larynx. An easy and safe endoscopic procedure seemed to be successful in the case described., (© 2015 Wiley Periodicals, Inc.)

Spinal angiolipomas are uncommon benign tumours composed of mature fatty tissue and abnormal vascular elements, most commonly found within the posterior spinal epidural space. Most tumours are located within the mid-thoracic spine; in contrast thoracolumbar junction and purely lumbar angiolipomas are rare. We report a case series of four spinal angiolipomas, including a thoracolumbar junction and a purely lumbar tumour., (Copyright © 2015 Elsevier Ltd. All rights reserved.)

Background: Extradural spinal angiolipomas are extremely rare benign neoplasms made up of mature lipocytes with abnormal blood vessels. Spinal angiolipomas represent only 0.14-1.2% of all spinal axis tumours., Case Presentation: A case of thoracic spinal extradural angiolipoma producing acute spinal cord compression in a 35-year old housewife is presented. Patient presented with sudden onset of lower limbs paralysis and urinary incontinence for 1 month after vaginal delivery. Patient was diagnosed as dorsal spine angiolipoma which was treated surgically with excellent outcome., Conclusions: Spinal angiolipomas are rare tumours but it is mandatory to include it in the differential diagnosis of the spinal extradural space occupying lesions. Pregnancy and vaginal delivery may suddenly exacerbate the condition. The best investigation to choose to diagnose these lesions is definitely magnetic resonance imaging of the spine. The aim of treatment of extradural angiolipomas of the spine is to resect the tumour in Toto surgically with no need of adjuvant therapy, surgery alone can lead to excellent outcome.

Despite their relatively frequent occurrence on the trunk and extremities, lipomas rarely present in the orbit. Rarer still are variants of lipoma such as fibrolipoma, myxoid lipoma, and angiolipoma. The authors report a 66-year-old woman who presented with a large, slowly growing tumor of the forehead and orbit. The case presentation conforms to the tenets of the Declaration of Helsinki and is HIPAA compliant. Clinical and radiographic evidence suggested a lipomatous type of tumor, and excisional biopsy revealed adipose proliferation with numerous small vessels and fibrin thrombi consistent with angiolipoma. The tumor was completely excised without ophthalmic sequelae or recurrence in 6 months of follow up. To the authors' knowledge, this tumor represents only the second reported case of this type of highly vascularized lipomatous lesion within the orbit. This case is a novel entity in the differential diagnosis of orbital tumors and demonstrates the value of total excision in such cases.

A 77-year-old Japanese woman was transported to a nearby hospital due to sudden abdominal pain and transient loss of consciousness. Abdominal computed tomography (CT) suggested hemoperitoneum and hepatic nodule. She was conservatively treated. Contrast-enhanced CT two months later revealed an increased mass size, and the enhancement pattern suggested the possibility of hepatocellular carcinoma (HCC). Under a clinical diagnosis of HCC, transcatheter arterial chemoembolization (TACE) was performed. A subsequent imaging study revealed that most of the lipiodol used for the embolization was washed out. Therefore, surgical resection was performed. Histologically, the nodule contained numerous inflammatory cells including small lymphocytes, plasma cells and macrophages. Notably, epithelioid granulomatous features with multinucleated giant cells were observed in both the nodule and background liver. Some of the multinucleated giant cells contained oil lipid. Among the infiltrating inflammatory cells, spindle-shaped, histiocytoid or myoid tumor cells with eosinophilic cytoplasm were found. The tumor cells were positive for Melan A and HMB45. The nodule contained many IgG4-positive plasma cells; these were counted and found to number 72.6 cells/HPF (range: 61-80). The calculated IgG4:IgG ratio was 33.2%. The nodule was finally diagnosed as previously ruptured inflammatory angiomyolipoma modified by granulomatous reaction after TACE.

A 6-year-old female spayed Shetland Sheepdog presented for evaluation of a subcutaneous mass over the right prescapular region. The mass had been cytologically diagnosed as a lipoma by the referring veterinarian 20 months prior, but had grown significantly and was very firm. CT scan of the mass was suggestive of neoplasia; however, the tissue of origin could not be determined. Histopathologic evaluation diagnosed infiltrative angiolipoma, and marginal resection of the tumor was performed. Infiltrative angiolipomas are benign but locally aggressive neoplasms uncommonly reported in veterinary medicine. This report correlates the clinical, CT, and histopathologic characteristics of an infiltrative angiolipoma., (© 2014 American College of Veterinary Radiology.)

A woman in her mid-50s presented with a 3-month history of upper abdominal pain. Initial examination using ultrasound was unremarkable and the patient was sent home. The patient returned 8 days later and CT imaging revealed intussusception as the cause of her symptoms. The involved bowel was surgically reduced and transected with the lead point found to contain a 3 cm mass. Histological examination revealed the mass to be an angiolipoma. To the best of our knowledge, this is the first reported case of adult jejunojejunal intussusception secondary to angiolipoma., (2015 BMJ Publishing Group Ltd.)

Angiolipomas of the spine are rare benign tumors commonly presenting with compressive myelopathy. The present report describes a case of spinal angiolipoma with thoracic mediastinal extension in a 50-year-old woman. She presented with a long-standing history of mid-back pain with progressive lower extremities weakness. An MRI showed a heterogeneously enhancing mass located in the posterior epidural space of the thoracic spine with mediastinal extension. Histopathological examination demonstrated features consistent with spinal angiolipoma. This report emphasizes the diagnosis and therapeutic management options of infiltrating spinal angiolipomas.