Manifestación clínica inicial de la lipomatosis epidural lumbar grado III de Naka: serie de casos / Initial clinical signs of Naka's grade III lumbar epidural lipomatosis: A case series

Introducción: La lipomatosis epidural espinal es una enfermedad infrecuente caracterizada por el sobrecrecimiento del tejido adiposo no encapsulado dentro del espacio epidural. Esto genera una estenosis del conducto espinal que puede provocar sintomatología compresiva. La presentación típica es insidiosa a lo largo de meses o años. El objetivo de este estudio fue realizar un análisis descriptivo de la manifestación clínica inicial en pacientes con lipomatosis epidural espinal grado III de Naka. Materiales y métodos: Estudio observacional retrospectivo en la Unidad de Patología Espinal de 4 instituciones, de 2010 a 2023. Se incluyó a pacientes >18 años, de ambos sexos, que acudieron por dolor lumbar con irradiación o sin irradiación, y presentaban lipomatosis lumbar Naka III en la resonancia magnética. Resultados: Se incorporó a 40 pacientes (edad promedio 62.5 años). El 75% era obeso, ninguno era fumador. El motivo de consulta más frecuente fue lumbalgia, con una mediana de evolución del dolor de 5.5 meses. Conclusiones:La lumbalgia fue la consulta más frecuente, con excepción del compromiso de L3-S1 que fue la lumbocruralgia. Los pacientes que sufrieron dolor más tiempo (>6 meses) eran más jóvenes y tenían un índice de masa corporal menor; sin embargo, no resultó estadísticamente significativo. Nivel de Evidencia: IV

Introduction: Spinal epidural lipomatosis (SEL) is a rare pathology characterized by the overgrowth of nonencapsulated adipose tissue within the epidural space. This generates spinal stenosis, which might result in compression symptoms. The typical presentation is insidious pain that lasts months or years. The objective of this study was to carry out a descriptive analysis of the initial clinical signs of patients with Naka's grade III lumbar SEL. materials and methods: Retrospective observational study in the Spinal Pathology Unit of 4 institutions, from 2010 to 2023. Patients over the age of 18, of both sexes, who consulted for low back pain with or without radiation and presented Naka's grade III lumbar lipomatosis on magnetic resonance imaging (MRI) were included. Results: We included 40 patients, with a mean age of 62.5 years; 75% were obese, there were no smokers. The most frequent reason for consultation was low back pain, with a median duration of 5.5 months. Conclusions: The most common reason for consultation was low back pain, with the exception of L3-S1 level involvement, which caused lumbar pain with radiation to the thigh. Patients with a longer period of pain (>6 months) were younger and had a lower BMI; although this was not statistically significant. Level of Evidence: IV

Clinical research progress of spinal epidural lipomatosis / 中国修复重建外科杂志

OBJECTIVE@#To review the clinical research progress of spinal epidural lipomatosis (SEL).@*METHODS@#The clinical studies on SEL at home and abroad in recent years were extensively reviewed, and the pathogenesis, clinical and imaging manifestations, and treatment status of SEL were summarized and analyzed.@*RESULTS@#SEL is a disease characterized by compression of the spinal cord and nerve roots due to abnormal accumulation of epidural adipose tissue in the spinal canal. Its prevalence and diagnosis rate are low and the pathogenesis is not fully understood. MRI is the most sensitive and specific diagnostic test for SEL. Surgical decompression and removal of excess adipose tissue are the only options for patients with acute SEL or those who have failed conservative management, and conservative management should be considered for other patients.@*CONCLUSION@#SEL is a rare disease and related research still needs to be improved. In the future, high-quality, multi-center and large-sample studies will be of great significance for evaluating the choice of treatment methods and effectiveness of SEL patients.

Nevo lipomatoso cutáneo superficial / Superficial cutaneous lipomatous nevus

RESUMEN El nevo lipomatoso cutáneo superficial es un nevo o hamartoma del tejido conectivo, idiopático, caracterizado por tumoraciones lobuladas del color de la piel, únicas o múltiples, con cierta predilección por la cintura pélvica; su aparición es poco frecuente. Histológicamente se destaca la presencia de células grasas maduras localizadas ectópicamente en la dermis. Se presentó un paciente de 40 años de edad con lesión en la piel de la región glútea derecha desde niño. Al examen dermatológico presentaba lesiones papulonodulares múltiples, de color de la piel, de tamaño variable, de consistencia blanda, localizadas en la nalga derecha. Se le realizó exéresis y biopsia de piel de lesión papulonodular de mayor tamaño, con diagnóstico histopatológico de nevo lipomatoso cutáneo superficial.

ABSTRACT A superficial cutaneous lipomatous nevus is an idiopathic connective tissue nevus or hamartoma characterized by single or multiple lobulated skin-colored tumors with a certain predilection for the pelvic girdle; its appearance is rare. Histologically, the presence of mature fat cells located ectopically in the dermis stands out. We present a 40-year-old male patient with a skin lesion of the right gluteal region since he was a child. On dermatological examination, he had multiple, skin-colored, papulonodular lesions of variable size and soft consistency located on the right buttock. Exeresis and skin biopsy of a larger papulonodular lesion were performed, with histopathological diagnosis of superficial cutaneous lipomatous nevus.

Lipomatosis intestinal: reporte de dos casos / Intestinal lipomatosis: Report of two cases

Resumen La lipomatosis intestinal es un término utilizado para describir una proliferación de tejido graso, histológicamente normal, en el tracto gastrointestinal. Se caracteriza por la presencia de tumores benignos, asintomáticos, de crecimiento lento y de origen subepitelial. En raras ocasiones, estos tumores pueden ser sintomáticos y asociarse a complicaciones importantes como obstrucción y sangrado gastrointestinal. Así, la lipomatosis intestinal es una patología rara, con pocos casos reportados en la literatura y ninguno registrado en Colombia. A continuación, presentamos dos casos evaluados y seguidos en nuestro servicio de Unión de Cirujanos S.A.S de la Universidad de Caldas. El primero es el caso de un hombre de 70 años con historia de episodios de dolor abdominal tipo cólico, asociados a distensión y diarrea posprandial. La endoscopia digestiva alta inicial reportó múltiples lesiones amarillentas, submucosas, en el duodeno, con diagnóstico histológico de lipomas. En este caso, se realizó una videocápsula endoscópica para determinar la distribución y las características de las lesiones presentes en todo el tracto gastrointestinal, así como para valorar la presencia de complicaciones. El otro caso es el de un hombre de 81 años, quien ingresó a la institución por lipotimia y sangrado rectal. Por tanto, se realizó una endoscopia alta y una colonoscopia, cuyos resultados fueron normales. Luego de ello, se efectuó una videocápsula endoscópica que mostró lesiones lipomatosas; una de ellas, con sangrado y angiodisplasia al lado. Estas lesiones se manejaron mediante enteroscopia de doble balón y terapia con argón plasma.

Abstract Intestinal lipomatosis is a proliferation of histologically normal fatty tissue in the gastrointestinal tract. It is characterized benign, asymptomatic, slow growing tumors with sub-epithelial origins. On rare occasions, they become symptomatic and can be associated with major complications such as gastrointestinal obstructions and bleeding. This rare pathology has been reported several times in the literature but not in Colombia prior to this study. We present two cases that were evaluated and followed up at the Unión de Cirujanos SAS and the University of Caldas in Manizales, Colombia. The first is a 70-year-old man who had a history of colic associated with bloating and postprandial diarrhea. The initial upper digestive endoscopy reported multiple yellowish, submucosal lesions in the duodenum. His histological diagnosis was lipomas. In this case, an endoscopic video capsule determined the distribution and characteristics of the lesions throughout the gastrointestinal tract and assessed complications. The second is an 81-year-old man who entered the institution due to lipothymia and rectal bleeding. Upper endoscopy and colonoscopy were normal, but a videocapsule endoscopy showed lipomatous lesions one of which was bleeding and had adjacent angiodysplasia. He was treated with double balloon enteroscopy and Argon plasma therapy.

Lipomatosis gástrica: reporte de un caso / Gastric lipomatosis: a case report

Resumen La lipomatosis gástrica es una enfermedad infrecuente caracterizada por múltiples lesiones tumorales benignas (lipomas), que acorde a su tamaño pueden producir una variedad de síntomas. Por lo general, la enfermedad es documentada incidentalmente en estudios imagenológicos que se realizan para estudiar otras enfermedades y los hallazgos patológicos contribuyen a tener una certeza en el diagnóstico de esta patología. Por el momento, no hay un tratamiento definido para las masas pequeñas y asintomáticas, mientras que para las masas grandes (> 3-4 cm) o sintomáticas se sugiere la resección quirúrgica de las lesiones.

Abstract Gastric lipomatosis is a rare disease characterized by multiple lipomas, benign tumors which can produce a variety of symptoms according to their size. In general, the disease is incidentally documented in imaging studies done to study other diseases. Pathological findings can contribute to the certainty of diagnosis. At the moment, there is no definite treatment for small, asymptomatic masses, but surgical resection is suggested for masses that are larger than 3 or 4 cm and for those that are symptomatic.

Caecal appendix lipomatosis in a pregnant patient mimicking acute appendicitis / Lipomatose do apêndice cecal em paciente grávida mimetizando apendicite aguda

ABSTRACT A 34-years-old pregnant woman admitted in the emergency unit complaining about worsening right iliac fossa pain for 2 days. Acute appendicitis was the suspected diagnosis. Laboratory exams were ordered and results were within normal limits for infectious and inflammatory aspects. Ultrasound scan revealed a pregnancy in course without alterations and a thickness of the appendix wall without inflammatory signs in the surrounding tissue. Because the suspicion of acute appendicitis remained, a magnetic resonance was done and confirmed the diagnosis of a cecal appendix lipomatosis.

RESUMO Paciente de 34 anos, do sexo feminino, grávida, chega ao pronto-socorro com queixa de dor na fossa ilíaca direita piorando nos últimos 2 dias com suspeita de apendicite aguda. Foram solicitados exames laboratoriais, que estavam dentro dos limites de normalidade para aspectos infecciosos e inflamatórios. Exame de imagem também foi solicitado, sendo a ultrassonografia o método de escolha, que revelou gravidez em curso sem alterações e espessura da parede do apêndice sem sinais inflamatórios. Ainda com suspeita de apendicite aguda, foi realizada ressonância magnética, confirmando a hipótese de lipomatose do apêndice cecal.

Incidental diagnosis of breast cancer in the pursuit of the treatment of intestinal obstruction

Intestinal lipomatosis is rare and often asymptomatic but can present with intestinal obstruction. Occasionally, metastatic breast cancer is identified in the ovary before a breast primary is discovered. We report the case of a 50-year-old woman diagnosed with synchronous intestinal obstruction due to lipomatosis, and incidental ovarian metastases from breast cancer. The patient presented with a 12-day history of nausea, diffuse abdominal pain, and constipation. An abdominal x-ray showed air-fluid levels, and computed tomography documented small bowel distention. An explorative laparotomy was performed, which revealed small bowel distention, an obstructive lesion of the ileocecal valve, three terminal ileum lesions, ascites, and heterogeneous ovaries. Right ileocolic resection and left oophorectomy were performed. The pathological diagnosis revealed lipomatous submucosal lesion of the ileocecal valve and ileum, and 17 lymph nodes, which were all negative for malignant cells. The oophorectomy revealed ovarian metastasis from breast carcinoma. Ascitic fluid was positive for malignant cells. Mammography and breast/axillary ultrasonography showed a solid nodule of the left breast, ductal carcinoma, and multiple enlarged left axillary lymph nodes, which were positive for neoplastic cells. Immunohistochemical evaluation showed hormonal receptor positivity and C-erb2 negativity. Breast magnetic resonance imaging showed a 14 mm left nodule and a positron emission tomography scan revealed 18F-FDG uptake in the left breast, left axillary lymph nodes, right ovary, and peritoneum. The tumor was staged as stage IV ductal breast carcinoma, cT1N1M1, Grade 2, Luminal B-like. The multidisciplinary oncological meeting proposed chemotherapy, and a re-staging breast MRI after chemotherapy, which showed a complete response. The patient started treatment with letrozole and remains disease-free 22 months after finishing chemotherapy.

Tenosynovial Bilateral Lipoma Arborescens of the Ankle in Adults / 대한족부족관절학회지

Lipoma arborescens or synovial lipomatosis is a rare disorder that is characterized by mature fat infiltration of the hypertrophic synovial villi, most frequently affecting the supra-patellar pouch of the knee. This paper presents a case of lipoma arborescens of the ankle joint bilaterally in an adult patient with involvement of both the intra-articular synovium and the synovial sheath of the tendons around the ankle.

Congenital infiltrating lipomatosis of face with seizures: a case report / 华西口腔医学杂志

Congenital infiltrating lipomatosis of the face is a rare disorder resulting from overgrowth of adipose tissues. This condition presents gradually with swelling along with age, hypertrophy of adjacent bones, and tooth abnormalities. This study reports a case of congenital infiltrating lipomatosis of face with seizures and reviews relevant literature on the etiology, clinical symptom, diagnosis, and treatment of this condition.

Extensive renal sinus lipomatosis in xanthogranulomatous pyelonephritis simulating liposarcoma

ABSTRACT Renal replacement lipomatosis is a condition characterized by varying degrees of renal parenchymal atrophy and perirenal fibrofatty proliferation secondary to chronic inflammation such as xanthogranulomatous pyelonephritis. In severe cases, imaging findings can be misdiagnosed as retroperitoneal liposarcoma.

Lipedematous Alopecia in an Asian Woman: Is It an Advanced Stage of Lipedematous Scalp?

Lipedematous alopecia (LA) is a rare disease entity that manifests as increased thickness of subcutaneous fatty tissue in the scalp with a variable degree of acquired, non-scarring alopecia. Although the pathogenesis of alopecia in LA is not clear, it is assumed that the increased thickness of subcutaneous fat retards hair growth by disturbing the peripheral microenvironment of the hair bulb. LA is clinically distinguishable from lipedematous scalp (LS) in that LS is not associated with any hair abnormalities, while LA is characterized by variable degree of hair loss. However, both LA and LS share increased scalp thickness. Here, we describe a rare case of LA on the frontal scalp of an Asian woman, further characterize the clinical and histologic features, and suggest applying an integrated diagnosis as lipedematous diseases of the scalp.

Nevus lipomatosus cutaneous superficialis

Abstract: Nevus lipomatosus cutaneous superficialis is a rare benign hamartomatous skin tumor characterized by dermal deposition of mature adipose tissue. Two clinical forms have been described (classical and solitary types). We describe a case of nevus lipomatosus cutaneous superficialis with a 13-year history of growth in a young woman who had a solitary skin-colored tumoral mass on the right buttock. Histopathological findings were typical and confirmed the diagnosis. In this case, the lesion was a skin-colored isolated mass, as described in the solitary type, but its localization and age of appearance were compatible with the classical type. The combination of simultaneous clinical findings of both types had not been published before.

Lipomatosis congénita infiltrante de la cara: reporte de un caso y revisión de la literatura / Congenital infiltrating lipomatosis of the face: case reporte and literature review

La lipomatosis congénita infiltrante de la cara (CLIF) es una enfermedad de origen congénito de baja prevalencia. Sus características clínicas son bien definidas. Éstas generan asimetrías faciales evidentes en los individuos que las padecen, las que pueden observarse clínicamente así como también en radiografías y estudios histopatológicos. El diagnóstico de lipomatosis se hace al nacimiento o a edades muy tempranas y su tratamiento también comienza cuando los pacientes son muy pequeños. Es de gran importancia el trabajo en equipo con cirujanos plásticos, cirujanos maxilofaciales, ortodoncistas, cirujanos dentistas y sicólogos. Este trabajo presenta el caso clínico de una niña chilena con diagnóstico de lipomatosis congénita infiltrante de la cara y el tratamiento que se le ha realizado hasta la fecha.

Congenital infiltrating lipomatosis of the face (CLIF) is a present at birth disease of low prevalence. Its clinical characteristics are well defined. They generate evident facial asymmetries in individuals who suffer from them which can be seen clinically as well as in X-rays or histopathological studies. Diagnosis of lipomatosis is made at birth or at very early stages and its treatment also starts when the patients are very young. It is of great importance the teamwork with plastic surgeons, maxillofacial surgeons, orthodontists, dental surgeons and psychologists. This paper shows the case of a Chilean girl with diagnosis of congenital infiltrating lipomatosis of the face and the treatment that she has received up to date.

Lumbar Disc Herniation Presented with Contralateral Symptoms

OBJECTIVE: This study aimed to unravel the putative mechanism underlying the neurologic deficits contralateral to the side with lumbar disc herniation (LDH) and to elucidate the treatment for this condition. METHODS: From January 2009 to June 2015, 8 patients with LDH with predominantly contralateral neurologic deficits underwent surgical treatment on the side with LDH with or without decompressing the symptomatic side. A retrospective review of charts and radiological records of these 8 patients was performed. The putative mechanisms underlying the associated contralateral neurological deficits, magnetic resonance imaging (MRI), electromyography (EMG), and the adequate surgical approach are discussed here. RESULTS: MRI revealed a similar laterally skewed paramedian disc herniation, with the apex deviated from the symptomatic side rather than directly compressing the nerve root; this condition may generate a contralateral traction force. EMG revealed radiculopathies in both sides of 6 patients and in the herniated side of 2 patients. Based on EMG findings and the existence of suspicious lateral recess stenosis of the symptomatic side, 6 patients underwent bilateral decompression of nerve roots and 2 were subjected to a microscopic discectomy to treat the asymptomatic disc herniation. No specific conditions such as venous congestion, nerve root anomaly or epidural lipomatosis were observed, which may be considered the putative pathomechanism causing the contralateral neurological deficits. The symptoms resolved significantly after surgery. CONCLUSION: The traction force generated on the contralateral side and lateral recess stenosis, rather than direct compression, may cause the contralateral neurologic deficits observed in LDH.

Cas rare de lipomatose généralisée (maladie de Launois-Bensaude) chez un sujet noir africain

La maladie de Launois-Bensaude (ou syndrome de Madelung) considérée comme rare est définie par l'accumulation de graisse sous-cutanée non-encapsulée surtout sur la partie supérieure du tronc et la racine des membres. Nous rapportons le premier cas rare de syndrome de Madelung décrit chez un patient noir africain. Il s'est agi d'un patient de 55 ans, éthylique chronique reçu pour tuméfaction sous-cutanées multiples, symétriques, généralisées. L'aspect dysmorphique du patient était frappant. Les principales localisations de ces tuméfactions étaient : cervicale, thoracique, abdominale, brachiale, crurale. Il y avait une adipomastie bilatérale. Une hyperuricémie a été retrouvée. Le scanner TAP montrait des masses lipomateuses diffuses et une stéatose hépatique. La résection chirurgicale ou la liposuccion peuvent être d'un intérêt thérapeutique associées aux mesures hygiéno-diététiques

Nevus lipomatosus cutaneous superficialis

Abstract We report a case of nevus lipomatosus cutaneous superficialis of Hoffman-Zurhelle (NCLS), with multiple lesions, in a ten-year-old child. The NLCS is considered rare. The classical clinical presentation is characterized by multiple skin-colored or yellowish papules and nodules, which can have a linear distribution. Histologically, it is characterized by the presence of mature ectopic adipocytes in the dermis. The main therapeutic option is surgical excision. The classical Nevus lipomatosus cutaneous superficialis is reported in this case.

Lipoma gigante do coxim adiposo bucal: relato de caso e revisão da literatura / Giant lipoma of the buccal fat pad: case report and literature review

INTRODUÇÃO: Lipomas são os tumores benignos mesenquimais mais comuns. Entretanto, são pouco frequentes na face, principalmente o tipo considerado gigante, derivado do coxim adiposo bucal. A literatura é escassa e provavelmente a sua incidência é subestimada. MÉTODO: Relato do caso de tratamento cirúrgico de lipoma gigante bucal e temporal recidivado, e revisão da literatura no Pubmed na língua inglesa e na Revista Brasileira de Cirurgia Plástica. RESULTADOS: O tumor, anatômica e morfologicamente, corresponde ao coxim adiposo bucal, foi totalmente retirado pelo acesso facial e temporal, sem sequelas ao nervo facial. Foram encontrados 31 casos relatados de lipoma de origem do coxim adiposo bucal, entretanto, vários casos foram encontrados e relatados sob outras denominações. DISCUSSÃO: O coxim adiposo bucal é maior e mais complexo que se conhecia, e várias patologias se derivam deste, sendo importante o diagnóstico diferencial do lipoma simples com o de células fusiformes e com o lipossarcoma, devido a sua extrema semelhança. CONCLUSÃO:Nas lesões lipomatosas da face, a possível origem no coxim adiposo bucal deve ser considerada. Um estudo amplo dessas lesões com a finalidade de uniformizar a terminologia e de determinar a sua real incidência deve ser realizado.

INTRODUCTION: Lipomas are the most common benign mesenchymal tumors. Nevertheless, they are infrequent in the face, particularly giant lipomas, which are derived from the buccal fat pad. The literature regarding these tumors is scarce and their incidence is likely underestimated. METHODS: We present a case report of surgical treatment of a relapsed giant buccal and temporal lipoma and review the related English literature in Pubmed and that in the Brazilian Journal of Plastic Surgery. RESULTS: The tumor, which anatomically and morphologically corresponded to the buccal fat pad, was completely excised by facial and temporal access without sequelae to the facial nerve. A total of 31 reported cases of lipoma originating from the buccal fat pad were found; however, several were found and reported under other names. DISCUSSION: The buccal fat pad is larger and more complex than assumed, and several pathologies are derived thereof, making the differential diagnosis of simple lipoma with fusiform cell lipoma and liposarcoma difficult due to their extreme similarities. CONCLUSION: In lipomatous lesions of the face, the possible origin in the buccal fat pad must be considered. An extensive study of these lesions with the purpose of standardizing the terminology and determining its real incidence must be performed.

A rare case of dysembryoplastic neuroepithelial tumor combined with encephalocraniocutaneous lipomatosis and intractable seizures / 소아과

Encephalocraniocutaneous lipomatosis (ECCL) is a rare neurocutaneous syndrome that affects ectomesodermal tissues (skin, eyes, adipose tissue, and brain). The neurologic manifestations associated with ECCL are various including seizures. However, ECCL patients very rarely develop brain tumors that originate from the neuroepithelium. This is the first described case of ECCL in combination with dysembryoplastic neuroepithelial tumor (DNET) that presented with intractable seizures. A 7-year-old girl was admitted to our center because of ECCL and associated uncontrolled seizures. She was born with right anophthalmia and lipomatosis in the right temporal area and endured right temporal lipoma excision at 3 years of age. Seizures began when she was 3 years old, but did not respond to multiple antiepileptic drugs. Brain magnetic resonance (MR) imaging performed at 8 and 10 years of age revealed an interval increase of multifocal hyperintense lesions in the basal ganglia, thalamus, cerebellum, periventricular white matter, and, especially, the right temporal area. A nodular mass near the right hippocampus demonstrated the absence of N-acetylaspartate decrease on brain MR spectroscopy and mildly increased methionine uptake on brain positron emission tomography, suggesting low-grade tumor. Twenty-four-hour video electroencephalographic monitoring also indicated seizures originating from the right temporal area. Right temporal lobectomy was performed without complications, and the nodular lesion was pathologically identified as DNET. The patient has been seizure-free for 14 months since surgery. Although ECCL-associated brain tumors are very rare, careful follow-up imaging and surgical resection is recommended for patients with intractable seizures.