Pancreatoduodenectomy due to lipomatous pseudohypertrophy of the pancreas / Pancreatoduodenectomia por pseudohipertrofia lipomatosa do pâncreas

ABSTRACT BACKGROUND: Lipomatous pseudohypertrophy of the pancreas, pancreatic lipomatosis, pancreatic steatosis, non-alcoholic fatty pancreatic disease, or fatty pancreas is an extremely rare disease, characterized by the organ enlargement and a localized or diffuse replacement of pancreatic acinar cells by mature adipose tissue, preserving the pancreatic ductal system and islets of Langerhans. AIMS: To report a rare case of lipomatous pseudohypertrophy of the pancreas in a symptomatic patient and the surgical treatment employed. METHODS: A 24-year-old male patient with weight loss (10 kilograms in 8 months), hyperglycemia, severe and recurrent acute abdominal pain, epigastric discomfort associated with nausea, vomiting, and jaundice for 40 days. Magnetic resonance imaging was performed, revealing an irregular lipomatous pseudohypertrophy of the pancreas, measuring 6.0 × 5.6 cm in the head, uncinate process, and part of the body of the pancreas. The pancreatic duct dilation was diffuse and irregular, associated with atrophy of the remnant parenchyma, particularly in the tail of the pancreas. The patient underwent pancreatoduodenectomy without total mesopancreas excision followed by pancreatojejunostomy. RESULTS: The postoperative course was uneventful, the length of stay in the ICU was two days, and the patient was discharged on the seventh postoperative day. CONCLUSIONS: The disease treatment depends on the signs and symptoms at presentation and a pancreatoduodenectomy is indicated in patients with severe and recurrent abdominal pain.

RESUMO RACIONAL: Pseudo-hipertrofia lipomatosa do pâncreas, lipomatose pancreática, esteatose pancreática, doença pancreática gordurosa não alcoólica ou pâncreas gorduroso é uma doença extremamente rara, caracterizada por aumento do órgão e substituição localizada ou difusa de células acinares pancreáticas por tecido adiposo, preservando o sistema ductal pancreático e as ilhotas de Langerhans. OBJETIVOS: Relatar um caso raro de pseudo-hipertrofia lipomatosa do pâncreas, em paciente sintomático e o tratamento cirúrgico empregado. MÉTODOS: Paciente do sexo masculino, 24 anos, com queixas de emagrecimento (10 quilos nos últimos 8 meses), hiperglicemia, dor abdominal aguda intensa e recorrente, desconforto epigástrico associado a náuseas, vômitos e icterícia há 40 dias. A ressonância magnética (RM) revelou pseudo-hipertrofia lipomatosa irregular do pâncreas, medindo 6,0 × 5,6 cm na cabeça, processo uncinado e parte do corpo do pâncreas. A dilatação do ducto pancreático era difusa e irregular, associada à atrofia do parênquima remanescente, principalmente na cauda do pâncreas. O paciente foi submetido à duodenopancreatectomia sem excisão total do mesopâncreas seguida de pancreatojejunostomia. RESULTADOS: O pós-operatório transcorreu sem intercorrências, o tempo de internação na UTI foi de 2 dias, com alta hospitalar no sétimo dia. CONCLUSÕES: O tratamento da doença depende dos sinais e sintomas na apresentação e a duodenopancreatectomia é indicada em pacientes com dor abdominal intensa e recorrente.

Doença de Madelung: abordagem cirúrgica combinada de lipectomia e lipoaspiração / Madelung's disease: combined surgical approach of lipectomy and liposuction

A doença de Madelung (DM) ou lipomatose simétrica múltipla é uma patologia caracterizada pelo acúmulo de tecido adiposo não encapsulado e depositado simetricamente ao redor do pescoço e tronco superior (tipo I - forma mais comum). Sua etiologia ainda é pouco esclarecida, porém apresenta evidente associação com o consumo crônico excessivo de bebidas alcoólicas. As deformidades físicas são o que levam o paciente a buscar serviço médico, juntamente com eventuais sintomas de acometimento cervical como redução de mobilidade e afecções respiratórias. O diagnóstico da lipomatose simétrica múltipla é clínico, podendo ser complementado com exame de imagem para afastar demais hipóteses diagnósticas e avaliar a extensão do acometimento. O tratamento pode ser realizado por duas modalidades: clínico ou cirúrgico (lipectomia ou lipoaspiração). Relata-se o caso de paciente com lipomatose simétrica múltipla tipo I abordado cirurgicamente com ambas as técnicas: lipectomia cervical e lipoaspiração abdominal. Paciente evoluiu de maneira satisfatória, com redução de queixas e sem recidivas até o presente momento.

Madelung's disease or Multiple Symmetric Lipomatosis is a condition characterized by the accumulation of unencapsulated adipose tissue deposited symmetrically around the neck and upper trunk (type I - most common form). Its etiology is still unclear, but it is clearly associated with chronic excessive consumption of alcoholic beverages. Physical deformities lead the patient to seek medical care, along with possible symptoms of cervical involvement such as reduced mobility and respiratory disorders. Multiple Symmetric Lipomatosis diagnosis is clinical and can be complemented with imaging to rule out other diagnostic hypotheses and assess the extent of involvement. Treatment can be performed in two ways: clinical or surgical (lipectomy or liposuction). We report the case of a patient with Multiple Symmetric Lipomatosis type I surgically treated with both techniques: cervical lipectomy and abdominal liposuction. The patient evolved satisfactorily, with a reduction in complaints and no recurrences so far.

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.

Lipoma Arborescente: uma causa de sinovite crônica? / Lipoma Arborescens: a cause of chronic synovitis?

O lipoma arborescente é uma causa incomum de lesão intra-articular que se apresenta como aumento de volume articular indolor, lentamente progressivo, que persiste por muitos anos e é acompanhado por derrames articulares intermitentes. O envolvimento de sítios extra-articulares é incomum, mas pode ocorrer em bainhas tendíneas e bursas. A ressonância magnética é o melhor exame para o diagnóstico, embora a biópsia sinovial possa ser necessária em alguns casos. Relatamos três casos com o objetivo de destacar o espectro clínico da doença, as características da imagem e a resposta ao tratamento imunossupressor.

Lipoma arborescens is an uncommon cause of intra-articular masses that presents as slowly progressive painless swelling of the joint, which persists for many years and is accompanied by intermittent effusions. Extra-articular site(s) involvement is unusual, but can occur in tendon sheaths and bursas. Magnetic resonance imaging is the best diagnostic exam, although synovial biopsy may be necessary. We report three cases in order to highlight the clinical spectrum and imaging features of the disease, so that early diagnosis and appropriate treatment can be given.

Discondrosteosis de Léri-Weill. Presentación de una familia afectada / Léri-Weill dyschondrosteosis. Presentation of an affected family

RESUMEN Fundamentación: La discondrosteosis de Léri-Weill, displasia ósea de origen genético que afecta la región mesomélica con acortamiento de las extremidades, provoca talla baja con extremidades cortas con deformidad de Madelung; esta enfermedad muestra un patrón de herencia autosómico dominante con alta penetrancia. Objetivo: Describir las deformidades de esta discondrosteosis de baja frecuencia con expresividad variable, que se presentó de la misma forma en todos los afectados de esta familia. Presentación de caso: Se reportó una familia con enfermos en tres generaciones con deformidad de Madelung de ambas muñecas y baja estatura de origen mesomélico, que se mantiene seguimiento en consultas de Genética Clínica y Ortopedia. Conclusiones: El examen físico y radiológico imprescindibles para llegar al diagnóstico clínico. El método clínico y la valoración multidisciplinaria resultaron de gran valor para definir esta enfermedad y poder brindar un adecuado asesoramiento genético a esta familia.

ABSTRACT Background: Léri-Weill dyschondrosteosis, bone dysplasia of genetic origin that affects the mesomelic region with shortening of the extremities, causes short stature with short extremities with Madelung deformity.This disease shows an autosomal dominant inheritance pattern with high penetrance. Objective: To describe the deformities of this low frequency dyschondrosteosis with variable expressivity which was presented in the same way in all those affected in this family. Case presentation: A family with sick members was reported in three generations with Madelung deformity of both wrists and short stature of mesomelic origin which is followed up in consultations of Clinical Genetics and Orthopedics. Conclusion: The essential physical and radiological examination to reach the clinical diagnosis. The clinical method and the multidisciplinary assessment were of great value to define this disease and to be able to provide adequate genetic counseling to this family.

Esteatose pancreática: um novo diagnóstico e desafio terapêutico na Gastroenterologia / Pancreatic steatosis: a new diagnosis and therapeutic challenge in gastroenterology

ABSTRACT Fat infiltration in the pancreas is called pancreatic steatosis and it has several synonyms such as pancreatic lipomatosis, non-alcoholic fatty pancreatic disease, lipomatous pseudohypertrophy, fatty replacement, fatty pancreas and fatty infiltration. Pancreatic steatosis describes a disease ranging from infiltration of fat in the pancreas to pancreatic inflammation, and development of pancreatic fibrosis. There are multiple aetiologies of this condition, such as metabolic syndrome, alcohol intake, viral infections, toxins, congenital syndromes, etc. Pancreatic steatosis is usually diagnosed by trans-abdominal ultrasound, computed tomography scan and magnetic resonance imaging. Fatty infiltration in pancreas may lead to pancreatitis, diabetes mellitus and may be a predisposing cause of pancreatic cancer. Now a day, pancreatic steatosis is a common incidental finding during abdominal ultrasonography for other reasons and is a new challenge in Gastroenterology. But there is no guideline for pancreatic steatosis till now. In this review article, we are trying to give an overall idea (aetiologies, diagnosis, management, clinical significances) on pancreatic steatosis.

RESUMO A infiltração de gordura no pâncreas é chamada de esteatose pancreática ou lipomatose pancreática e tem vários sinônimos tais como: doença pancreática gordurosa não alcoólica, pseudo hipertrofia lipomatosa, reposição gordurosa, pâncreas gorduroso e infiltração gordurosa. A esteatose pancreática descreve uma doença que vai desde a infiltração de gordura no pâncreas até a inflamação pancreática com o desenvolvimento de fibrose pancreática. Existem múltiplas causas dessa condição, como síndrome metabólica, ingestão de álcool, infecções virais, toxinas, síndromes congênitas, etc. A esteatose pancreática é geralmente diagnosticada por ultrassom trans-abdominal, tomografia computadorizada ou ressonância magnética. A infiltração gordurosa no pâncreas pode levar à pancreatite e pode ser uma causa predisponente ao câncer de pâncreas. Hoje em dia, a fibrose pancreática é um achado incidental comum durante a ultrassonografia abdominal realizada por outras razões e é um novo desafio na Gastroenterologia. Mas não há diretriz para esteatose pancreática até agora. Neste artigo de revisão, objetivamos dar uma ideia geral sobre esteatose pancreática.

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.

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.

Colonic intussusception as a rare manifestation of colonic lipoma: case report

ABSTRACT Introduction: Colonic lipomas are low-risk lesions that require endoscopic or surgical management depending on their size and symptoms. However, diagnosing a lipomatous lesion in the colon is a difficult task, as the clinical or imaging findings point to a low prevalence of these lesions and a higher frequency in the female sex. The laparoscopic approach is the current method of choice as it represents fewer traumas and, therefore, a faster clinical recovery. Case presentation: 43-year-old female patient, Caucasian, from the city of Bogotá, housewife, without any medical history. She presented with acute abdominal pain associated with tarry stools. Diagnostic imaging tests were performed, finding colo-colonic intussusception, originated in a lipomatous lesion located in the transverse colon. This patient underwent an enlarged laparoscopic right hemicolectomy. Discussion: This case describes how colonic intussusception constitutes a preoperative complication that requires surgical management since its diagnosis considering the associated mortality rates. Conclusion: The analysis of this case is part of the scientific evidence indispensable to improve the diagnostic and therapeutic approach to colonic intussusception, as local and international literature have not addressed this condition sufficiently.

RESUMEN Introducción. Los lipomas en el colon son lesiones con pocos riesgos de malignización que, según su tamaño y sintomatologia, pueden requerir manejo endoscópico o quirúrgico. Sin embargo, es difícil establecer el diagnóstico de una lesión lipomatosa en el colon: los hallazgos clínicos o imagenológicos solo describen que la prevalencia de estas lesiones es baja y que es una patología común en el género femenino. Debido a que el abordaje laparoscópico causa menores traumas y por tanto la recuperación clínica es más rápida, en la actualidad suele elegirse esta opción. Presentación de caso. Paciente femenino de 43 años, raza blanca, procedente de Bogotá D.C., ama de casa y sin antecedentes médicos. La sintomatología que presentó fue dolor abdominal de instauración aguda asociado a deposiciones melénicas, por lo que fue llevada a toma de imágenes diagnósticas donde se documentó intususcepción colocolónica originada en una lesión lipomatosa y localizada en el colon transverso. Se le practicó hemicolectomía derecha ampliada por vía laparoscópica. Discusión. A partir de este caso se describe cómo la intususcepción colónica constituye una complicación preoperatoria que demanda la realización de un manejo quirúrgico desde el mismo diagnóstico dado el margen de los porcentajes de mortalidad. Conclusión. El análisis del presente caso constituye parte de la evidencia científica indispensable para mejorar el abordaje diagnóstico y terapéutico de la intususcepción colónica que la literatura nacional e internacional no ha tratado suficientemente.

Diffuse Intestinal Lipomatosis presenting as Intussusceptions: A Case Report

Introduction: Gastrointestinal tract is a uncommon site forlipomas. Intestinal lipomas are most common benign nonepithelial intestinal tumors, found incidentally. Symptomaticdiffuse submucosal intestinal lipomatosis is a rare entity. Here,we present a rare case of intestinal submucosal lipomatosis,which presented as intestinal obstruction.Case report: On laparotomy, intussusception of a segment ofgut was identified and sent for histopathological examination.On gross examination, intussusception of a part of gutsegment with distally gangrenous gut segment was identified.On cutting open, multiple soft, yellowish polypoidal growthsidentified. On histopathology, multiple submucosal lipomaswere identified.Conclusion: Diffuse intestinal lipomatosis is a rare conditionand can lead to intestinal obstruction and should be kept as adifferential diagnosis in a case of intussusceptions.

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.

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.

Madelung disease (multiple symmetric lipomatosis)

Madelung disease or multiple symmetric lipomatosis (MSL) is a rare entity among the overgrowth syndromes. It is characterized by painless non-encapsulated and symmetric fatty deposits in the neck, torso, mammary, and abdominal areas, and in the upper and lower limbs. The etiology of the disease is still unknown. Chronic alcohol consumption may play a role in adipocyte hyperplasia in genetically susceptible individuals. Besides the overgrowth of adipose tissue, patients with MSL present features of metabolic syndrome. Patients seek medical attention usually for esthetic reasons. We present the case of a middle-aged man who sought the outpatient clinic complaining of bulging masses in the posterior upper part of the thorax, the occipital area, and the neck. The masses grew over a period of 2 years. The physical examination and imaging study revealed the presence of symmetric lipomatosis. A two-step surgical treatment was undertaken for the excision of the lipomatous tissue. The postoperative outcome was uneventful with satisfactory esthetic results.

Adipose metaplasia of the thyroid associated with Hashimoto's thyroiditis with nodular hyperplasia and oncocytic cells adenoma: case report and literature review / Metaplasia adiposa de tireoide associada à tireoidite de Hashimoto com hiperplasia nodular e adenoma de células oncocíticas: relato de caso e revisão da literatura

ABSTRACT Lesions involving adipose tissue and the thyroid gland are usually rare. In the present report, adipose metaplasia is associated with Hashimoto's thyroiditis with nodular hyperplasia and oncocytic adenoma in a 40-years-old female patient, which is one of the first reported cases of this association. The main clinical finding is the diffuse goiter, and the differential diagnosis must be performed to exclude the possibility of malignancies. The treatment is performed through total thyroidectomy. Thus, it is important the physicians be aware of this pathology with a view to a more effective clinical reasoning and to exclude more serious possibilities.

RESUMO Lesões que envolvem tecido adiposo e glândula tireoide costumam ser raros. No presente relato, a metaplasia adiposa associou-se à tireoidite de Hashimoto com hiperplasia nodular e adenoma de células oncocíticas em uma paciente de 40 anos, sexo feminino, sendo um dos primeiros casos relatados dessa associação. O principal achado clínico é o bócio difuso, e o diagnóstico diferencial deve ser realizado com o intuito de excluir malignidades. O tratamento é feito por meio de tireoidectomia total. Dessa forma, é importante que o médico conheça essa patologia a fim de um raciocínio clínico mais eficaz, excluindo possibilidades mais graves.

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.

Extrapleural solitary fibrous tumor with uncommon histology: a clinicopathologic analysis of 7 cases / 中华病理学杂志

Objective@#To investigate the clinicopathologic characteristics, immunophenotypes, and differential diagnostic features of extra-pleural solitary fibrous tumor (SFT) with uncommon histology.@*Methods@#Seven cases of extra-pleural SFT with uncommon histology were collected during January 2015 and December 2016 in Zhejiang Provincal People′s Hospital; the clinical and radiologic features, histomorphology, immunophenotype and prognosis were analyzed. EnVision method was used for immunohistochemical staining of STAT6, CD34 and other differential diagnosis associated markers.@*Results@#There were five male and two female patients, age from 23 to 54 years (mean=39 years). Three tumors were located in the soft tissue of head and neck, two in trunk subcutaneous soft tissue, one in sella region, and one in the kidney. Grossly the tumors ranged from 0.4 to 8.0 cm (mean=3.1 cm). Microscopically, all three head and neck cases resembled giant cell angiofibroma/giant cell subtype SFT, and one case showed sheet-like pattern of the multinucleated syncytial cells, creating a biphasic arrangement similar to myofibroma. Both truncal tumor resembled lipomatous type SFT, with one similar to dermatofibrosarcoma protuberans and the other to atypical spindle cell lipomatous tumor. The sella tumor showed morphology of a conventional SFT with high grade sarcomatous transformation. The renal tumor demonstrated a malignant SFT with entrapped benign renal tubules, mimicking a biphase synovial sarcoma or a malignant mixed epithelial and stromal tumor. By immunohistochemistry, all seven SFTs showed diffuse and strong nuclear reactivity to antibody against STAT6.@*Conclusions@#Extra-pleural SFTs show a significant heterogeneity of morphology and biological behavior which could cause differential confusion.Careful attention to its characteristic histomorphology with the use of STAT6 immunohistochemistry can help distinguish this tumor from its many mimickers.

Pelvic lipomatosis: urodynamic characters and clinical significance / 中华泌尿外科杂志

Objective To study the urodynamic characters of pelvic lipomatosis(PL) and its clinic significance.Methods From Sep.2013 to Feb.2016,7 patients,including 6 males and one female,were diagnosed as PL by image examinations and related lower urinary tract syndrome.Their mean age was 46 years,ranged from 30 to 52 years.All the patients(study group) have normal renal function.However,4 patients were noticed bilateral hydronephrosis.Other patients with similar LUTS but were confirmed as normal by image and urodynamic examinations were concluded in control group,which was paired designed to match the study group in visiting time,gender,age,and number of patients.The uroflowmetry and pressure/flow cystometry were performed in all patients.The urodynamic data of two groups including maximum flow rate (Qmax),time to maximum flow,post-voiding residual (PVR),first sensation capacity,first desire to void capacity,cystometric capacity,intravesical pressure at terminal of filling phase,compliance,detrusor pressure at maximum flow,detrusor opening pressure,bladder outlet obstruction index (BOOI) and bladder contractility index (BCI),were retrospectively analyized.Results There were no significant different between study aud control groups respect to Qmax [(18.71 ± 10.31) ml/min vs.(13.29 ± 6.55) ml/min],time to maximum flow [(6.71 ±2.50)s vs.(7.43±2.76)s],PVR [(28.14±27.81)ml vs.(3.14± 3.48) ml],first sensation capacity[(138.86 ± 77.01) ml vs.(142.29 ± 89.84) ml],first desire to void capacity [(203.71 ± 131.09) ml vs.(216.57 ± 72.20) ml],cystometric capacity [(271.0 ± 151.95) ml vs.(323.29± 92.87)ml],i ntravesical pressure at terminal of filling phase [(30.29 ± 16.45)cmH2O vs.(18.71 ± 9.57) cmH2 O],and compliance [(90.53 ± 126.46) cmH2O/ml vs.(129.57 ± 136.25) cmH2O/ml].The study group was significant higher than control group in terms of pressure at maximum flow [(69.57 ±25.06) cmH2O vs.(33.14 ± 11.63) cmH2O,P =0.004],opening pressure [(42.57 ± 22.16)cmH2O vs.(18.00±13.18) cmH2O,P=0.027],BOOI [(34.00±15.92)vs.(16.50±5.96),P=0.030] and BCI [(133.17 ± 27.37) vs.(87.67 ± 20.16),P =0.008],respectively.Moreover,the BCI of PL patients with hydronephrosis were significant higher compare with PL patients without hydronephrosis [(155.67 ±14.98) vs.(110.67 ± 1 1.37),P =0.014].Conclusions The urodynamic characters of PL were increased pressure at maximum flow and opening pressure,which implied obstruction.

Two Cases of Multiple Symmetric Lipomatosis in Women with Alcoholism / 대한피부과학회지

Madelung's disease, also known as multiple symmetric lipomatosis (MSL) is a rare disease characterized by the presence of multiple, symmetric, loose adipose tissues distributed around the neck, shoulder, back or chest. MSL is different from simple obesity, which is characterized by the presence of well-distributed total body fat. It is rarely reported in Asia and most commonly seen among middle-aged men. Although the etiology of MSL is unknown, the disease is associated with alcoholism in 60% to 90% of patients. The diagnosis is usually made on the basis of the history, and clinical appearance. Also, imaging such as CT or MRI could confirm the diagnosis. Herein, we experienced an uncommon case of MSL in middle-aged women with alcoholism.

Relato de caso: doença de Madelung / Case report: Madelung's disease

A síndrome de Madelung ou também conhecida por lipomatose simétrica múltipla (LSM) é uma lipodistrofia benigna rara que se caracteriza por múltiplas massas adiposas não encapsuladas. Ainda de etiologia desconhecida, existe a suspeita de uma possível herança autossômica dominante. Está fortemente associada ao abuso de álcool (60-90% dos casos). Apresenta curso clínico variável, de crescimento rápido com posterior estabilização ou progressão lenta. Pode ser assintomática ou causar sintomas de compressão de estruturas mediastinais e do trato aerodigestivo. O tratamento de escolha é cirúrgico (AU)

Madelung syndrome, also known as multiple symmetric lipomatosis (MSL), is a rare benign lipodystrophy that is characterized by multiple non-encapsulated adipose masses. Although of unknown etiology, there is a suspected autosomal dominant inheritance. It is strongly associated with alcohol abuse (60-90% of cases). It presents a variable clinical course, of fast growth with later stabilization or slow progression. It may be asymptomatic or cause symptoms of compression of mediastinal structures and aerodigestive tract. The treatment of choice is surgical (AU)