Spinal dysraphism in exstrophy: a single-center study of a 39-year prospective database.

OBJECTIVE: The two main objectives of this study were to explore the rate of spinal dysraphism within bladder and cloacal exstrophy and to analyze the relationship between spinal dysraphism surgery, including timing of spinal dysraphism surgery, with urological and neurological outcomes. METHODS: A prospectively maintained IRB-approved database of pediatric exstrophy patients treated from 1982 to 2021 was retrospectively reviewed for patients with spinal dysraphism. Spinal dysraphism was categorized into the following 7 subtypes: lipoma-based closed defect, myelomeningocele, meningocele, diastematomyelia, myelocystocele, low-lying conus with tethered cord/fatty filum, and sacral bony defect. Other factors assessed included patient demographic characteristics, type of spinal dysraphism procedure, reoperation, complication, presence of other neurological problems (e.g., hydrocephalus, Chiari malformation), neurological status, and urological function. RESULTS: Analysis revealed that 114/1401 patients had coexisting spinal dysraphism. Of these 114, sufficient records including type of dysraphism were available for 54. Spinal dysraphism was most common within cloacal exstrophy (83.3% [45/54 patients]), followed by cloacal exstrophy variants (9.3% [5/54]), classic bladder exstrophy (3.7% [2/54]), and classic bladder exstrophy variants (3.7% [2/54]). Within spinal dysraphism, lipoma-based closed defects (63.0% [34/54]) and low-lying conus with tethered cord/fatty filum (11.1% [6/54]) were most common. Hydrocephalus and Chiari malformation occurred in 24.1% (13/54) and 11.1% (6/54) of patients. All 13 patients with hydrocephalus underwent shunt placement. Among those who underwent neurosurgical intervention, the complication rate for spinal dysraphism was 14.6% (7/48). Motor function data were available for 41 patients and revealed that motor function declined for 2/41 (4.8%) patients and improved for 6/41 (14.6%) after neurosurgery. There was no statistical difference in lower-extremity motor outcome related to timing of neurosurgery and exstrophy closure. CONCLUSIONS: The authors have reported the surgical management and outcomes of patients with exstrophy and coexisting spinal dysraphism (n = 54). In 54 patients, spinal dysraphism was most common in the subset of patients with cloacal exstrophy (83.3%). Lipoma-based closed defects (63.0%) and low-lying conus with tethered cord/fatty filum (11.1%) were the most common, and the rates of hydrocephalus and Chiari malformation were 24.1% and 11.1%, respectively. There was no difference in lower-extremity motor outcome related to timing of neurosurgery and exstrophy closure.

A 14-year retrospective study focusing on clinical and morphological features of oral cavity lipomas: A review of main topics.

BACKGROUND: Lipomas are benign soft tissue neoplasms frequently found in the human body. Head and neck lipomas are relatively uncommon, accounting for 1 to 4% of benign lesions at this location. This 14-year retrospective study analyzed the clinical-pathological features of cases of oral cavity lipomas and their histopathological variants seen at a single oral pathology referral center. MATERIAL AND METHODS: Data on age, sex, anatomical location, clinical diagnosis, and histological subtypes were collected from all cases microscopically diagnosed as lipoma. Three previously trained oral pathologists re-evaluated hematoxylin/eosin-stained slides of all selected cases. RESULTS: Among 7,861 oral and maxillofacial lesions diagnosed at the service, 95 (1.2%) were lipomas or their histopathological variants. There was a predominance of female patients (n = 65; 68%); the mean age at diagnosis was 58.8 years (±13.56). We found the following histological subtypes: conventional lipoma, fibrolipoma, spindle cell lipoma, sialolipoma, osteolipoma, chondrolipoma, and intramuscular lipoma. The buccal mucosa was the most affected site. Conventional lipoma and fibrolipoma were the most commonly diagnosed histological variants. Although most lipomas are asymptomatic, large lipomas can occur, reaching a diameter of 4 cm. CONCLUSION: The present study reinforces the importance of careful clinical and histopathological examination in order to obtain an accurate diagnosis and to ensure appropriate treatment.

Cerebellopontine Angle and Internal Auditory Canal Lipomas: Case Series and Systematic Review.

OBJECTIVES/HYPOTHESIS: Cerebellopontine angle (CPA) and internal auditory canal (IAC) lipomas are rare, benign tumors comprising 0.08% of all intracranial tumors and can be mistaken for other, more common lesions of the CPA/IAC such as vestibular schwannoma. The purpose of this study was to review the literature and assess the evolution of CPA/IAC lipoma diagnosis and management. In addition, we present 17 new lipomas, matching the largest known case series of this rare tumor. STUDY DESIGN: Retrospective case series and systematic review. METHODS: Systematic review of the literature was performed using PubMed and Google Scholar. References from identified articles were also reviewed to identify potential additional manuscripts. Manuscripts and abstracts were reviewed to identify unique cases. For the case series, the charts of all CPA/IAC lipoma patients seen at a single institution from 2006-2019 were manually reviewed. Logistic regression and chi-squared analysis were performed where appropriate. RESULTS: A total of 219 unique lipomas have been reported in the literature, including 17 presented in this study. Surgical management has been performed in 46% of cases and has been conducted less often in recent decades, likely due to improved radiographic diagnostic capabilities and understanding of surgical outcomes. Surgical management is associated with worse neurologic outcomes (P = .002) and has become less common in recent decades. Although growth is unlikely, it has been demonstrated in patients into their 30s. CONCLUSIONS: Accurate radiographic diagnosis is imperative for appropriate patient management, as CPA/IAC lipomas should typically be managed through observation and serial imaging whereas vestibular schwannomas and other CPA/IAC lesions may require microsurgical or radiosurgical intervention depending on growth and symptomatology. Laryngoscope, 131:2081-2087, 2021.

Tumors of the labial mucosa: a retrospective study of 1045 biopsies

BACKGROUND: To investigate the relative frequency of localized mucosal swellings of the upper and lower labial mucosa, the clinical-pathological diagnosis agreement and whether patient's age and gender and tumor's site and size may raise the suspicion of neoplasm. MATERIAL AND METHODS: Retrospective analysis was performed on upper or lower labial mucosal tumors, histopathologically diagnosed between 2009-2018. The diagnostic categories developmental/reactive tumors, benign and malignant neoplasms were associated with patient's age and gender and tumor's site and size; clinical-pathological diagnosis agreement was, also, evaluated. RESULTS: Overall, 1000 (95.7%) developmental/reactive tumors, 35 (3.3%) benign and 10 (1%) malignant neoplasms were found. Upper/lower lip tumor ratio was 0.14:1. The diagnostic category was significantly associated with age (p < 0.0001), site (p < 0.0001) and diameter (p < 0.0001). Age ≥60 years, tumor's location on the upper lip and diameter >1cm were independent predictors for neoplasms. Patients presenting 2 or 3 of these variables were 20.2 times (p < 0.0001) or 33.6 times (p < 0.0001), respectively, more likely to have a neoplasm. Complete/partial agreement between clinical and pathological diagnosis was seen in 96.3% of the cases. CONCLUSIONS: Most lip tumors involve the lower lip and are reactive, but upper lip tumors measuring > 1 cm in patients ≥ 60 years have significantly higher probability to be neoplasms

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Paratesticular tumors. A clinicopathological study from a single tertiary hospital in North India.

OBJECTIVE: Paratesticular tumors (PTT) are rare and form a heterogenous group, ranging from benign to malignant high grade sarcomas. This study was undertaken to describe the clinicopathological spectrum of PTTs received over a 20-year period. METHODS: All primary and secondary PTTs diagnosed from 2000 to 2019 in the pathology department of a tertiary care hospital in North India were retrospectively reviewed. Gross, histopathological features and immunohistochemistry (IHC) findings were correlated with clinical details. RESULTS: A total of 169 intra-scrotal tumors were diagnosed during the study period, out of which there were 30 PTTs (in 27 patients) comprising 17.75%. Age range was 4 to 85 years (median 58 years). Benign PTTs were the commonest (n = 21, 70%), followed by metastasis to the paratesticular region (n = 6, 20%) and then primary malignant PTTs (n = 3, 10%). The commonest benign PTT was lipoma (n = 16, 76.19%), followed by adenomatoid tumor (n = 3, 14.28%) with one case each (4.76%) of cellular angiofibroma and hemangioma. Among primary malignant PTT, there were two cases of rhabdomyosarcoma, and one case of biphasic malignant mesothelioma. Metastatic tumors included four cases of prostatic adenocarcinoma, and one case each of pancreatic signet ring cell carcinoma and clear cell renal cell carcinoma. CONCLUSION: PTTs show a wide clinicopathological spectrum. Benign PTTs are commoner than malignant PTTs. Meticulous grossing and histopathological examination supplemented by IHC is essential for an accurate diagnosis of this heterogenous class of tumors, which influences the role of adjuvant therapy and patient prognosis.

Clinical management of lipomas in dogs under primary care in the UK.

Lipomas are relatively common and biologically benign masses of mesenchymal origin consisting of adipocytes. This study reports benchmark data on the clinical management and outcomes of lipomas in dogs under UK primary veterinary care. The study used a cross-sectional analysis of cohort clinical data from dogs that were under veterinary care at practices participating within VetCompass from January 1, 2013 to December 31, 2013. Descriptive and analytic statistics characterised the clinical management and outcomes following presumptive lipoma diagnosis. The study included 2765 lipoma cases from 384 284 dogs under UK veterinary care during 2013. Diagnostics included fine needle aspirate in 1119 (40.5 per cent) cases, biopsy in 215 (7.8 per cent) cases and diagnostic imaging in 11 (0.4 per cent) cases. Overall, 525 (19.0 per cent) cases were managed surgically. Of the surgical cases, 307 (58.5 per cent) solely had mass removal whilst 218 (41.5 per cent) included another procedure during the same surgical episode. A surgical drain was placed during surgery in 90 (17.1 per cent) cases. Wound breakdown was reported in 14 (2.7 per cent) surgical procedures. Wound infection followed surgery in 11 (2.1 per cent) dogs. The findings provide veterinarians with an evidence base that benchmarks how lipoma cases are currently managed in the UK, but these results do not necessarily reflect optimal management or best practice.

The clinical and radiological features of cisternal and pericallosal lipomas.

PURPOSE: To demonstrate the clinical and radiological features of pericallosal and cisternal lipomas and to compare these two groups in terms of radiological and clinical features. METHODS: This retrospective study included 23 patients diagnosed with pericallosal and cisternal lipoma between 2010 and 2017. The patients comprised 12 females and 11 males with a mean age of 38.8 years (range 12-69 years). Magnetic resonance imaging was applied to all the patients. Computed tomography (CT) was also performed in 10 patients. RESULTS: 9 lesions were pericallosal and 14 were cisternal (7 were right quadrigeminal cistern, 3 were left quadrigeminal cistern, 1 was interpeduncular cistern, 1 was left cerebellopontine cistern, 1 was right cerebellopontine cistern, and 1 was supracerebellar cistern). The mean size of the lesions was 18.7 ± 10 mm. The shape of the lesions was curvilinear in 10 patients (43.5%) and tubulonodular in 13 patients (56.5%). The mean CT density of the lesions was - 87.7 ± 25.6 HU. The size of pericallosal lipomas (mean 27.5 mm) was statistically significantly larger than that of cisternal lipomas (mean 13.1 mm) (p = 0.001). No statistically significant difference was determined between the two groups in terms of lesion shape (p = 0.478), gender (p = 0.707), age (p = 0.639), and symptoms (p = 0.084). CONCLUSIONS: In this study, the incidence of pericallosal lipomas was 0.011% and the incidence of cisternal lipomas was 0.017%. Although rare, the knowledge of the clinical and radiological features of pericallosal and cisternal lipomas will play an important role in the accurate diagnosis and follow-up of the patient.

Prevalence of benign osseous lesions of the spine and association with spinal pain in the general population in whole body MRI.

BACKGROUND: Benign osseous lesions of the spine are common but precise population prevalence estimates are lacking. Our study aimed to provide the first population-based prevalence estimates and examine association with back and neck pain. MATERIALS AND METHODS: We used data from the population-based Study of Health in Pomerania (SHIP). Whole-body MRI examinations (1.5 Tesla: T1, T2, and TIRM weightings) were available from 3,259 participants. Readings of the spinal MRI images were conducted according to a standardized protocol by a single reader (JS). The intra-rater reliability was greater than Kappa values of 0.98. Pain measures included the seven-day prevalence of spine pain and neck pain, and average spine pain intensity due to spine pain during the past three months. RESULTS: We found 1,200 (36.8%) participants with at least one osseous lesion (2,080 lesions in total). Osseous lesions were less common in men than in women (35.5% vs 38.9%; P = .06). The prevalence of osseous lesions was highest at L2 in both sexes. The prevalence of osseous lesions increased with age. Up to eight osseous lesions were observed in a single subject. Hemangioma (28%), and lipoma (13%) occurred most often. Sclerosis (1.7%), aneurysmal bone cysts (0.7%), and blastoma (0.3%) were rare. Different osseous lesions occurred more often in combination with each other. The association with back or neck pain was mostly negligible. CONCLUSION: Osseous lesions are common in the general population but of no clinical relevance for spinal pain. The prevalence of osseous lesions varied strongly across different regions of the spine and was also associated with age and gender. Our population-based data offer new insights and assist in judging the relevance of osseous lesions observed on MRIs of patients.

Oral benign neoplasms: A retrospective study of 790 patients over a 14-year period / Neoplasias benignas orales: estudio retrospectivo de 790 patients durante un periodo de 14 años

Introduction and objective: Oral benign neoplasms (OBNs) exhibit some features that can guide the professionals to the correct diagnosis and best treatment. Through retrospective studies, medical records can be reviewed to better describe a given population and, furthermore, help clinicians in routine practice. In this context, the objective of this paper was to analyze the cases of OBNs of an oral pathology referral department, from 2003 to 2017, in order to better understand their epidemiological and clinicopathological characteristics. Methods: A total of 8355 histopathological reports were analyzed. Lesions diagnosed as OBNs were selected and the following variables were recorded: gender, age, histological type of the lesion, anatomical location, rate and pattern of growth, type of base, color, symptomatology and diagnostic hypotheses on clinical examination. Results: OBNs represented 9.4% of all lesions diagnosed. The most frequent histopathological types were fibroma (39.9%), papilloma (22%), fibroblastoma (13.1%), lipoma (10.2%) and hemangioma (6.1%). Overall, most cases affected females (n = 518; 65.6%) and in the fifth decade of life (n = 148; 18.7%). The oral mucosa was the most common site (n = 265; 33.5%). The most common features of each OBN were also highlighted. Conclusion: The most common OBNs were fibroma, papilloma, fibroblastoma, lipoma and hemangioma. Overall, the OBN presented common clinical features; however, in particular cases, there are some characteristics that can lead the professionals to the correct diagnosis. Nevertheless, in general, histopathological analysis must be performed to confirm diagnosis. Intraosseous tumors and large lesions may require imaging tests to help diagnosis

Introducción y objetivos: Las neoplasias benignas orales (NBO) presentan características clínico-patológicas específicas que pueden guiar al profesional al diagnóstico correcto y a un mejor tratamiento. Los estudios retrospectivos son uno de los principales recursos utilizados para conocer la situación actual de una población determinada y estimar sus necesidades para la implementación y el mantenimiento de los servicios de salud. En este contexto, el objetivo de este trabajo fue analizar los casos de NBO de un servicio de referencia en patología oral, de 2003 a 2017, para comprender mejor sus características epidemiológicas y clínico-patológicas. Métodos: se analizaron un total de 8355 informes histopatológicos. Se seleccionaron las lesiones diagnosticadas como NBO y se registraron las siguientes variables: sexo, edad, tipo histológico de la lesión, ubicación anatómica, índice y patrón de crecimiento, tipo de base, color, sintomatología e hipótesis diagnósticas en el examen clínico. Resultados: Las NBO representaron el 9,4% de todas las lesiones diagnosticadas. Los tipos histopatológicos más frecuentes fueron fibroma (39,9%), papiloma (22%), fibroblastoma (13,1%), lipoma (10,2%) y hemangioma (6,1%). También fueron verificados el perfil de los pacientes y las presentaciones clínicas de estas lesiones. Conclusiones: Las NBO más comunes fueron fibroma, papiloma, fibroblastoma, lipoma y hemangioma. En general, las NBO presentaron características clínicas comunes; sin embargo, en casos particulares, existen algunas características que pueden llevar al profesional al diagnóstico correcto. Sin embargo, de forma general, el análisis histopatológico debe hacerse para confirmar el diagnóstico. Los tumores intraóseos y las lesiones de gran tamaño pueden requerir exámenes de imagen para ayudar al diagnóstico

Multicentre retrospective study of 97 cases of intraoral lipoma.

BACKGROUND: Lipomas are benign neoplasms derived from adipose tissue that are unfrequently found in the oral cavity. The objective of this study is to identify the main clinicopathological characteristics of this tumour in a case series of oral lipomas (OL) in a population from Spain and Brazil. MATERIAL AND METHODS: A multicentre retrospective observational study was conducted in collaboration with the Oral Pathology Unit of the São Paulo State University (UNESP), São José dos Campos, in Brazil, and the Oral Medicine and Pathology Units of the University of Santiago de Compostela (USC) and the University of the Basque Country (UPV/EHU) in Spain. RESULTS: This multicentre series consists of a total of 97 of OL cases, 31 (32%) of which correspond to Spain, and 66 (68%) to Brazil with an overall prevalence of 0.33%. The average age of the patients is 56.44 years old, with a range of 10-92 (SD = 15.52). Buccal mucosa was the most frequent location (42.3%). Cases from Spain coexisted with other lesions in a significant number of cases, 12.9%, vs Brazil, 1.5% (P = 0.018). According to the progression time, Brazil has an average of 60.35 months (CI: 27.20-93.51), compared to 5.41 months in Spain (CI: 3.17-7.65) (P = 0.022). Average size is 12.55 mm (SD = 11.06), ranging from 2 to 75 mm. A positive correlation is also seen between the increase in the lesion size and a greater progression time (CC = 0.367; P = 0.008). CONCLUSIONS: Correct differential diagnosis of the clinical lesion is key, followed by a good histopathological study, to achieve the final diagnosis.

A review of forehead lipomas: Important tips for the training surgeon.

BACKGROUND: Forehead swellings can be caused by a variety of different pathologies, including lipomas. These benign lesions are composed of mature fat cells and often result in a poor cosmetic appearance causing significant psychosocial distress. Surgical removal is therefore frequently requested and they are often listed on a training local anaesthetic list. Thus, it is essential that trainees understand the nature of these lesions, their anatomical location and management, as well as differential diagnoses. METHODS: A review of the available literature on forehead lipomas and their management was performed. Searches of both MEDLINE and EMBASE were conducted, with all full text articles published in English being included for analysis. RESULTS: 14 papers were included for analysis. Forehead lipomas are slow growing lesions with 100% presenting as a solitary swelling. They are more prevalent in males with mean age at presentation being 48 years. Their aetiology is unknown, however, blunt trauma has been postulated to contribute to their formation. Differential diagnoses include cysts and extension of intracranial lesions. Surgical intervention is the only recognised treatment, with a transverse, forehead-crease incision most commonly being adopted under local anaesthetic. Exploration beneath the frontalis muscle is often required in order to achieve their excision. CONCLUSION: Important differential diagnoses must be excluded prior to attempted surgical excision of forehead lipomas. Surgeons must also be aware that the correct anatomical plane for these lesions is often beneath the frontalis muscle.

Oral benign neoplasms: A retrospective study of 790 patients over a 14-year period.

INTRODUCTION AND OBJECTIVE: Oral benign neoplasms (OBNs) exhibit some features that can guide the professionals to the correct diagnosis and best treatment. Through retrospective studies, medical records can be reviewed to better describe a given population and, furthermore, help clinicians in routine practice. In this context, the objective of this paper was to analyze the cases of OBNs of an oral pathology referral department, from 2003 to 2017, in order to better understand their epidemiological and clinicopathological characteristics. METHODS: A total of 8355 histopathological reports were analyzed. Lesions diagnosed as OBNs were selected and the following variables were recorded: gender, age, histological type of the lesion, anatomical location, rate and pattern of growth, type of base, color, symptomatology and diagnostic hypotheses on clinical examination. RESULTS: OBNs represented 9.4% of all lesions diagnosed. The most frequent histopathological types were fibroma (39.9%), papilloma (22%), fibroblastoma (13.1%), lipoma (10.2%) and hemangioma (6.1%). Overall, most cases affected females (n=518; 65.6%) and in the fifth decade of life (n=148; 18.7%). The oral mucosa was the most common site (n=265; 33.5%). The most common features of each OBN were also highlighted. CONCLUSION: The most common OBNs were fibroma, papilloma, fibroblastoma, lipoma and hemangioma. Overall, the OBN presented common clinical features; however, in particular cases, there are some characteristics that can lead the professionals to the correct diagnosis. Nevertheless, in general, histopathological analysis must be performed to confirm diagnosis. Intraosseous tumors and large lesions may require imaging tests to help diagnosis.

Lipomatous Soft-tissue Tumors.

Lipomatous soft-tissue tumors are the most common neoplasms encountered by physicians. They range from benign lipomas to high-grade liposarcomas. Unplanned excisions of sarcomas are commonly due to the presumptive diagnosis of lipoma and can be avoided by understanding their diagnostic magnetic resonance imaging appearance. Magnetic resonance images should be obtained for all soft-tissue masses that are deep to fascia or those >5 cm in subcutaneous tissue. Atypical lipomatous tumors present as large deep fatty masses and have a propensity for local recurrence and a small risk of malignant transformation. Well-differentiated liposarcomas are histologically identical to atypical lipomatous tumors but have a markedly worse prognosis secondary to their anatomic location. Masses that lack isointense signal to subcutaneous fat on MRI may represent a sarcoma and require a biopsy before definitive treatment. Large deep lipomatous masses and liposarcomas should be sent to a sarcoma referral center for definitive treatment.

Intraneural lipomas: institutional and literature review.

BACKGROUND: Adipose lesions of nerve can be envisioned as a spectrum ranging from intraneural/extraneural lipomas to lipomatosis of nerve (LN). We have noticed that intraneural lipomas are not as a homogenous group as previously thought and demonstrate differences which have clinical implications. To better understand intraneural lipomas, we conducted a search of cases at our institution and published cases in the world's literature. MATERIALS AND METHODS: Mayo Clinic's database was searched between years 1994-2018. Published cases were identified using PubMed and Google Scholar databases. Following terms were used: intraneural lipoma, lipoma and nerve, lipoma and neuropathy, lipofibroma and nerve, fibrolipoma and nerve and neural lipoma as well as lipofibroma and fibrolipoma alone. Cases that could be clearly identified as intraneural lipomas by the location of the lipoma within the epineurium were included for analysis. These cases were then sub-classified as encapsulated intraneural lipomas or hybrid intraneural lipomas (demonstrating features of both intraneural/extraneural lipomas and LN) based on their characteristics. RESULTS: We identified 12 cases at our institution (8 encapsulated, 4 hybrid) and 24 published cases (21 encapsulated, 3 hybrid). The most commonly affected nerve was median both at our institution and in the published cases. Encapsulated cases were found to be relatively easy to resect. Hybrid cases demonstrated variable degree of interdigitating fat between the fascicles and were relatively difficult to resect. CONCLUSION: Intraneural lipomas exist as two separate entities with distinct clinical implications. Although rare, this should be taken in account when planning surgery. Terminology should be clarified to prevent ambiguity and confusion.

Adipose tumors of the heart.

BACKGROUND: Primary cardiac adipose tumors are rare. There are two distinct pathologically defined entities that represent this tumor type: lipoma and lipomatous hypertrophy of the interatrial septum (LHIS). We present a single-center experience with these tumors and demonstrate that the location may not correspond to the pathologic diagnosis. METHODS: A retrospective review of a prospectively collected cardiac surgery database from January 1990 to July 2016 identified 254 cases of surgically treated primary cardiac tumors at our Institution. Of these, 06/254 (2%) were primary adipose tumors. RESULTS: In 3/6 (50%) cases, patients were asymptomatic or had symptoms referable to other known intracardiac lesions. Five patients (83%) had preserved ventricular function. In 4/6 cases (67%), the tumor was identified preoperatively. All patients presented in New York Heart Association functional class ≤2. Pathologic diagnosis of LHIS was made in 5/6 cases (83%), with 2/5 LHIS (40%) located in the interatrial septum. A bovine pericardial patch was utilized for reconstruction following tumor resection in 3/6 cases (50%). Mean cardiopulmonary bypass time was 88 ± 43 min. All the patients tolerated the procedure well without any postoperative complications. CONCLUSIONS: Primary cardiac adipose tumors are responsible for a small portion of all primary heart tumors. Surgical resection provided excellent outcomes, and did not affect cardiac performance, in spite of the need for extensive resections. LHIS was identified in locations other than the interatrial septum and was usually symptomatic.

Pulmonary thromboembolic events in patients with congenital lipomatous overgrowth, vascular malformations, epidermal nevi, and spinal/skeletal abnormalities and Klippel-Trénaunay syndrome.

OBJECTIVE: Patients with Klippel-Trénaunay syndrome (KTS) and congenital lipomatous overgrowth, vascular malformations, epidermal nevi, and spinal/skeletal abnormalities (CLOVES) syndrome have central phlebectasia and enlarged persistent embryonic veins that are often incompetent and prone to thromboembolism. The purpose of the study was to determine the presence of phlebectasia and the incidence of symptomatic pulmonary embolism (PE). METHODS: A retrospective review was conducted of patients referred to the Vascular Anomalies Center at our institution during a 21-year period who were diagnosed with KTS and CLOVES syndrome. Of these, the patients who had PE were screened for thromboembolic risk factors in addition to phlebectasia and the presence of persistent embryonic veins. Treatment outcomes following subsequent endovascular and medical therapies were reported. RESULTS: A total of 12 KTS patients of 96 (12.5%) and 10 CLOVES syndrome patients of 110 (9%) suffered PE. Fourteen patients (64%) developed PE after surgery or sclerotherapy. All of the patients had abnormally dilated central or persistent embryonic veins; 20 patients were treated with anticoagulation (1 died at the time of presentation, and no information was available for 1) after PE, and 14 (66%) patients underwent subsequent endovascular treatment. Five patients developed recurrent PE despite anticoagulation. Two of the patients died of PE. No patients treated with endovascular closure of dilated veins had subsequent evidence of PE. CONCLUSIONS: Patients with KTS and CLOVES syndrome are at high risk for PE, particularly in the postoperative period.

Intramucosal lipomas of the colon implicate Cowden syndrome.

Intramucosal lipomas are rare and easily overlooked by pathologists, despite their diagnostic significance for Cowden syndrome (PTEN hamartoma tumor syndrome), an inherited multiorgan cancer syndrome. Only 25-35% of patients harbor identifiable PTEN mutations, thus clinical features, like intramucosal lipomas, remain the mainstay of diagnosis. The significance and diagnostic approach to intramucosal lipomas have not been thoroughly addressed in the literature. Intramucosal lipomas are mimicked by pseudolipomatosis coli, an artifactual mucosal gas infiltration from endoscopic insufflation. This differential was investigated by morphology and S-100 immunohistochemistry. Twenty-five colonic intramucosal lipomas were identified from 176 archival gastrointestinal lipomas from 1998 to 2017. Controls included 40 submucosal lipomas and 30 pseudolipomatoses. S-100 immunohistochemistry on all 95 lesions confirmed delicate fat vacuole membranous and nuclear S-100 staining in lipomas absent from pseudolipomatoses. Differentiating morphology between intramucosal lipoma and pseudolipomatosis, respectively, included consistently large, regular fat vacuoles (92% vs 7%), associated spindle cells (80% vs 0%), and mucosal lymphoid aggregate involvement (12% vs 80%). Of the 25 intramucosal lipomas, five patients (20%) had confirmed Cowden syndrome (four with PTEN mutations). In four of these Cowden patients, the intramucosal lipoma was the index diagnostic lesion. Three (12%) intramucosal lipoma patients had additional clinical features associated with Cowden syndrome, but did not meet the diagnostic criteria. Sporadic-type intramucosal lipomas were identified in 17 patients (68%) without evidence of Cowden syndrome, including three with normal PTEN genetic testing. No distinguishing endoscopic or pathologic polyp features were identified between sporadic and syndromic intramucosal lipomas. These data provide evidence that intramucosal lipomas are important harbingers of Cowden syndrome, making up approximately one-third of this series, the largest in the literature. We also show for the first time that two-thirds of intramucosal lipomas are sporadic. Gastrointestinal pathologists, gastroenterologists, and geneticists should increase their awareness of this subtle but diagnosable lesion strongly associated with Cowden syndrome.

Hepatic lipomas and myelolipomas in subsistence-harvested bowhead whales Balaena mysticetus, Alaska (USA): a case review 1980-2016.

We describe a case series of benign hepatic fatty tumors in 10 subsistence-harvested bowhead whales. Microscopic features included lipomatous and myelolipomatous masses. Extensive atrophy and/or destruction of hepatic parenchyma was not observed. No other significant disease was present except in an animal with unrelated chronic pleuritis. Based on our longitudinal case series (1980-2016) which identified 1-2 hepatic lipomas and myelolipomas in landed whales annually at Barrow, Alaska (USA), since 2012, hepatic lipomas and myelolipomas are occasionally seen in hunter-harvested bowhead whales. A conservative estimate for the percentage of bowhead whales with hepatic fatty tumors in landed whales in Barrow from 2012 to 2016 was 6% (7/111). The pathogenesis and exact cell origin of these benign fatty tumors in bowhead whales is undetermined. Assessment of further cases is warranted to better define the tissue distribution and pathogenesis of these tumors in bowhead whale liver.