Terapia descongestiva como complemento a la cirugía de anastomosis linfáticovenosas y transferencia de linfonodos vascularizados: Una revisión de la literatura / Decongestive therapy as an adjunct to lymphatic-venous anastomosis surgery and vascularized lymph node transfer: A review of the literature

Resumen Introducción: El linfedema es una enfermedad inflamatoria crónica que afecta cerca de 250 millones de personas en el mundo. El tratamiento tradicional es la terapia descongestiva. Últimamente, existe la opción de complementar el tratamiento tradicional con procedimientos quirúrgicos fisiológicos como anastomosis linfáticovenosas y transferencia de linfonodos vascularizados. Sin embargo, la evidencia del uso de la terapia descongestiva en los cuidados pre y posoperatorios en estas cirugías es limitada. Objetivo: Evaluar el uso de terapia descongestiva como complemento a la cirugía de linfedema mediante anastomosis linfáticovenosas y transferencia de linfonodos vascularizados. Materiales y Método: Se realizó una revisión de la literatura en las siguientes bases de datos: Cochrane, Pubmed y Google académico, utilizando los siguientes términos mesh: "anastomosis, surgical", "lymphedema", "perioperative care", "microsurgery", "rehabilitation", "therapy", "lymph nodes", "bypass", "lymphedema and microsurgery". Se incluyó aquellos artículos que describían el uso de la terapia descongestiva en los cuidados pre- y posoperatorios. Resultados: Se identificó un total de 201 artículos y 12 fueron incluidos en el análisis. La evidencia reporta que las terapias más usadas en el cuidado pre- y posoperatorio son compresión, drenaje linfático manual y tratamientos personalizados. Sin embargo, la mayoría de los autores hace una descripción vaga de las terapias mencionadas. Discusión y Conclusión: La evidencia respecto al uso de terapia descongestiva como tratamiento complementario es débil. Los expertos recomiendan su uso, sin embargo, se necesitan futuras investigaciones que describan el uso de cada uno de sus componentes como complemento de procedimientos quirúrgicos fisiológicos para el manejo del linfedema.

Background: Lymphedema is a disease that affects about 250 million people around the world. The traditional treatment is decongestive therapy. In the past years, there is the option to complementing the traditional treatment with physiological surgical procedures such as lymphatic-venous anastomosis (LVA) and vascularized lymph node transfer (VLNT). However, the evidence for the use of decongestive therapy in pre- and post-operative care in these surgeries is limited. Aim: To evaluate the use of decongestive therapy as a complement to lymphedema surgery such a lymphatic-venous anastomosis and transfer of vascularized lymph nodes. Materials and Method: A literature review was carried out in the following databases: Cochrane, Pubmed and Academic Google, using the following mesh terms: "anastomosis, surgical", "lymphedema", "perioperative care", "microsurgery", "rehabilitation", "therapy", "lymph nodes","bypass", "lymphedema and microsurgery". "Those articles that described the use of decongestive therapy in pre- and post-operative care were included. Results: 201 articles were identified and 12 were included in the analysis. The evidence reports that the most used therapies in pre- and post-operative care are compression, manual lymphatic drainage and personalized treatments. However, most of the authors give a vague description of the mentioned therapies. Discussion and Conclusion: The evidence regarding the use of decongestive therapy as a complementary treatment is weak. Experts recommend its use; however, future research is needed to describe the use of each of its components as a complement to physiological surgical procedures for the management of lymphedema.

Signet ring cells in carcinomatous lymphangitis due to gastric adenocarcinoma

Abstract Cutaneous metastases are rare. They usually present as nodules or tumors. Diagnosis is based on histopathological examination and prognosis is unfavorable. This report describes the case of a female patient, 72 years old, with surgically treated gastric antrum adenocarcinoma. Pathology showed poorly differentiated adenocarcinoma with signet ring cells. It evolved with bone involvement, lymph node enlargement in the inguinal region, and skin infiltration in the lower limbs, abdomen, and root of the upper limbs. Skin biopsy demonstrated signet ring carcinoma embolizing the dermal and hypodermic vessels and invasion of adipose tissue, confirming carcinomatous lymphangitis. Carcinomatous lymphangitis is the cutaneous and subcutaneous lymphatic invasion by tumor cells. Cutaneous metastasis is relatively uncommon and presents mainly as cutaneous or subcutaneous nodules, and more rarely as inflammatory lesions. The present case reports carcinomatous lymphangitis associated with gastric cancer.

Algunos indicadores de la inmunidad celular y humoral en pacientes con linfangitis / Some indicators of cellular and humoral immunity in patients with lymphangitis

Introducción: La linfangitis es la inflamación de los vasos linfáticos producida por gérmenes patógenos, caracterizada por su recurrencia y el compromiso de su sistema inmune. Es frecuente en Cuba. Objetivo: Evaluar algunos indicadores de la inmunidad celular y humoral en pacientes con linfangitis. Métodos: Se realizó un estudio descriptivo, prospectivo y analítico en 75 pacientes divididos en tres grupos: sin linfangitis (referencia), con linfangitis en un primer episodio y con linfangitis recidivante; todos atendidos en el Instituto Nacional de Angiología y Cirugía Vascular. Las variables estudiadas fueron: edad, sexo, color de la piel y algunos parámetros de la inmunidad celular y humoral. Se utilizaron las pruebas no paramétricas chi cuadrado y t de Student para comparar los grupos entre sí. Resultados: Se observó un predominio de sexo femenino (n= 47, 62,7 por ciento); de edades superiores a los de 40 años (n= 61, 81,3 por ciento) y del color de piel blanca (n= 37, 49,3 por ciento). La obesidad, la insuficiencia venosa crónica y la Diabetes Mellitus fueron los factores de riego más frecuentes. El grupo con linfangitis recidivante, con respecto a los otros grupos, presentó alteraciones en la inmunidad humoral con concentraciones incrementadas (p = 0,007) de todas las inmunoglobulinas. No hubo variaciones significativas en la inmunidad celular. Conclusiones: Las alteraciones encontradas en la inmunidad celular y humoral de los pacientes con linfangitis, tanto en la primera crisis como en la recidiva, no son suficientes para sugerir que pudieran influir en los procesos sépticos asociados a esta afección(AU)

Introduction: Lymphangitis is the inflammation of the lymphatic vessels produced by pathogenic germs and characterized by its recurrence and the compromise of the immune system. It is frequent in Cuba. Objective: To evaluate some indicators of cellular and humoral immunity in patients with lymphangitis. Methods: A descriptive, prospective and analytical study was carried out in 75 patients divided into three groups: without lymphangitis (reference), with lymphangitis in a first episode, and with recurrent lymphangitis; all attended at the National Institute of Angiology and Vascular Surgery. The variables studied were: age, sex, color of the skin and some parameters of cellular and humoral immunity. The non-parametric chi square and Student's t tests were used to compare the groups among each other. Results: A predominance of females was observed (n= 47, 62.7 percent); ages over 40 years (n= 61, 81.3 percent) and white skin color (n= 37, 49.3 percent). Obesity, chronic venous insufficiency and diabetes mellitus were the most frequent risk factors. The group with recurrent lymphangitis, with respect to the other groups, presented alterations in humoral immunity with increased concentrations (p= 0.007) of all immunoglobulins. There were no significant variations in cellular immunity. Conclusions: The alterations in the cellular and humoral immunity of the patients with lymphangitis, both in the first crisis as in the recidive, are not enough to suggest that they may impact in the septic processes associated with this pathology(AU)

Antimoniato de meglumine perilesional en leishmaniasis cutánea con falla terapéutica sistémica: serie de casos / Perilesional meglumine antimonate in cutaneous leishmaniasis with systemic therapeutic failure: series of cases

En Bolivia, los medicamentos utilizados para el tratamiento de la leishmaniasis cutánea son los antimoniales, de aplicación sistémica en dosis/kg peso, los cuales provocan efectos adversos por la aplicación en largos periodos y grandes volúmenes. La aplicación perilesional de antimonio tiene similar eficacia terapéutica que la sistémica. Sin embargo, no se cuenta con información documentada respecto a la eficacia del tratamiento perilesional en pacientes con falla terapéutica, posterior al tratamiento sistémico. El objetivo de esta serie de casos fue evaluar el tratamiento perilesional con glucantime, en pacientes con leishmaniasis cutánea y falla terapéutica, posterior a un primer ciclo de tratamiento sistémico con antimoniales. El estudio se realizó con once pacientes con leishmaniasis cutánea con falla terapéutica, posterior a la administración de un primer ciclo de tratamiento sistémico con glucantime procedentes de la zona tropical de Bolivia. Se consideró como falla terapéutica la persistencia de la lesión y presencia de parásitos obtenidos de los bordes de la lesión. La intervención perilesional consistió en la aplicación día por medio de glucantime en cinco sesiones. La inoculación se realizó sobre el borde de la lesión y la dosificación del medicamento se calculó multiplicando el área de la lesión por el factor 0.008. Todos los pacientes presentaron dolor local durante el momento de inoculación del medicamento, así como también presentaron un ligero agrandamiento del área de la lesión después de la primera aplicación del medicamento En las siguientes inoculaciones se observó la reducción progresiva del área de la lesión hasta su completa cicatrización.

In Bolivia, the drugs used for the treatment of cutaneous leishmaniasis are the antimonials by systemic application in doses/kg/weight, which cause adverse effects due to the applications in long periods and large volumes. The perilesional application of antimony has similar therapeutic efficacy than the systemic one. However, there is no documented information regarding the efficacy of perilesional treatment in patients with therapeutic failure after systemic treatment. The aim of this series of cases was to evaluate the perilesional treatment with Glucantime, in patients with cutaneous leishmaniasis and therapeutic failure, after a first cycle of systemic treatment with antimonials. The study was conducted with eleven patients with cutaneous leishmaniasis and therapeutic failure, after the administration of a first cycle of systemic treatment with Glucantime. From the tropical zone of Bolivia. The persistence of the lesion and the presence of parasites at the edge of the lesion were considered as therapeutic failure. The perilesional intervention consisted in the application past one day of glucantime in five sessions. The inoculation was performed on the edge of the lesion and the dosage of the medication was calculated by multiplying the area of the lesion by the factor 0.008. All the cases presented local pain during the time of inoculation of the drug, as well as a slight enlargement of the area of the lesion after the first inoculation of the drug. In the following inoculations the progressive reduction of the area of the lesion was observed until its complete healing.

Lifangitis esclerosante no venérea del pene. Presentación de 2 casos y revisión de la literatura / Non-venereal sclerosing lymphangitis of the penis. Cases presentation and literature review

La linfangitis esclerosante no venérea del pene es una lesión benigna, en la mayoría delos casos autolimitada, que afecta a los hombres en su periodo de mayor actividad sexual, entre los 20 y 45 años de edad aproximadamente, aunque puede presentarse fuera de este rango de edad.1­3 A pesar de que se han reportado casos desde hace más de 60 años, no se ha esclarecido del todo su fisiopatología, siendo la teoría más aceptada la relacionada con traumatismos locales repetidos por relaciones sexualesvigorosas y/o masturbación con obstrucción linfática superficial secundaria.

Non-venereal sclerosing lymphangitis of the penis is a benign lesion, in most cases self-limited, affecting men in their period of greatest sexual activity, between approximately 20 and 45 years of age, although it may occur outside this age range.1-3 Although cases have been reported for more than 60 years, their physiopathology has not been fully clarified, with the most accepted theory being that related to repeated local trauma due to vigorous sexual intercourse and/or masturbation with secondary superficial lymphatic obstruction.

El sistema inmune en los linfedemas y linfangitis de los miembros inferiores / The immune system in lymphedema and lymphangitis of the lower limbs

El sistema inmune es el mecanismo de resistencia del organismo ante las infecciones. El sistema linfático juega un importante papel en el control fisiológico del fluido tisular y en la iniciación de la respuesta inmune. Nos proponemos describir la función de los componentes celulares del sistema inmune en las linfopatías de miembros inferiores no asociadas a cánceres tales como el linfedema y la linfangitis.Se revisaron las bases de datos PubMed, MedLine, SciELO, Clinical Key, Liliacs, Ebsco y artículos científicos publicados en revistas cubanas entre 2000-2015. Los estudios recientes muestran que el crecimiento de nuevos vasos linfáticos es una característica distintiva de las reacciones inflamatorias agudas y crónicas que caracterizan a las linfopatías, mediado por un incremento en el drenaje del fluido fuera del vaso y de células inflamatorias, así como de la modulación de las respuestas inmunes. Es apremiante continuar investigando, específicamente en lo concerniente al comportamiento de la inmunidad humoral y celular en los pacientes que padecen de linfedema o linfangitis, pues no se encontraron trabajos que aborden de manera específica la posible relación entre ambos. Se sugiere que las subpoblaciones de células T son un componente crítico en la respuesta celular inflamatoria crónica y subaguda en las linfopatías. La comprensión de la función reguladora del fluido linfático en la respuesta inflamatoria puede dar un importante paso en el desarrollo de tratamientos que puedan bloquear el inicio o la progresión de las consecuencias anómalas de las lesiones linfáticas(AU)

The immune system is the body´s mechanism of resistance to infections. The lymph system plays an important role in the physiological control of the tissue fluid and in the onset of the immune response. We intended to describe the function of the cell components of the immune system in the cancer-unrelated lymphopathies of the lower limbs such as lymphedema and lymphangitis. To this end, Pubmed, Medline, Scielo, Clinical Key, Liliacs, Ebsco and scientific articles published in Cuban medical journals from 2000 to 2015 were reviewed. The recent studies show that the growth of new lymphatic vessels is a distinctive characteristic of the acute and chronic inflammatory reactions of lymphopathies, mediated by increase of the fluid drainage outside the vessel and of inflammatory cells as well as the immune response modulations. It is urgent to continue studying this topic, mainly the behavior of the humoral and cell immunity in patients suffering from lymphedema or lymphangitis, since no research papers dealing with the possible relation between both aspects were found. It is suggested that the T-cell subpopulations are a key component of the chronic and sub-acute inflammatory response in lymphopaties. Hence, understanding of the regulating function of the lymph fluid in the inflammatory response may represent an important step in the development of therapies that might block the onset or progression of the anomalous consequences of lymphatic injures(AU)

Paracoccidioidomycosis: acute-subacute clinical form, juvenile type

Abstract: The authors report aspects of paracoccidioidomycosis, acute-subacute clinical form, juvenile type, in a 19-year-old female patient. Paracoccidioidomycosis, juvenile type, classically occurs in young patients, both sexes, with lymphoma-like aspects as initial presentation. However, following the natural history of the disease the lymph nodes assume patterns of infectious disease, as an abscess and fistulae. Systemic dissemination of the disease can occur and lethality and morbidity are significant in this clinical presentation.

Prevalence and Epidemiological Factors Involved in Cellulitis in Korean Patients With Lymphedema

OBJECTIVE: To evaluate the prevalence and associated factors involved in cellulitis with lymphangitis among a group of Korean patients who were being treated for lymphedema. We present our epidemiologic research and we also report a systematic review of these types of cases. METHODS: This was a retrospective medical record study among 1,246 patients diagnosed with lymphedema. The study was carried out between January 2006 and December 2012 at the Kosin University Gospel Hospital and Seoul National University Bundang Hospital. Cases were examined for onset time, affected site, seasonal trend, and recurrence pattern of lymphedema, lymphangitis, and cellulitis. We also evaluated the history of blood-cell culture and antibiotic use. RESULTS: Ninety-nine lymphedema patients experienced complications such as cellulitis with accompanying lymphangitis. Forty-nine patients had more than two recurrences of cellulitis with lymphangitis. The incidence and recurrence of cellulitis with lymphangitis were significantly higher in the patients with lower-extremity lymphedema. There was a significant trend toward higher cellulitis prevalence in the lower-extremity lymphedema group according to the time of lymphedema onset. Among the cellulitis with lymphangitis cases, 62 cases were diagnosed through blood-cell culture; 8 of these 62 cultures were positive for β-hemolytic streptococci. CONCLUSION: The prevalence rate of cellulitis with lymphangitis in patients with lymphedema was 7.95%, and the prevalence of recurrent episodes was 3.93%. Especially, there was high risk of cellulitis with lymphangitis after occurrence of lower-extremity lymphedema with passage of time. Lymphedema patients should be fully briefed about the associated risks of cellulitis before treatment, and physicians should be prepared to provide appropriate preventive education.

A Case of Lower Extremity Lymphedema in an Endstage Cancer Patient Complicated by Persistent Lymphorrhea / 대한피부과학회지

Secondary lymphedema occurs when normal lymphatics suffer a significant insult such as cancer, radiation therapy, surgery, trauma, or infection. Recurrent infections such as cellulitis, lymphangitis, and cutaneous lymphorrhea are significant complications in lymphedematous sites. A 58-year-old man with endstage colon cancer was referred to our department with a skin lesion and persistent discharge on both legs. On histopathological examination, numerous dilated lymphatic channels which were D2-40 positive were seen in the dermis. Herein, we report an interesting case of lower extremity lymphedema in an endstage cancer patient complicated with persistent cutaneous lymphorrhea.

Carcinomatosis linfática y embolismo tumoral pulmonar / Lymphangitic Carcinomatosis and Pulmonary Tumor Embolism

La linfangitis carcinomatosa es un trastorno relativamente poco común caracterizado por la infiltración difusa de los vasos linfáticos pulmonares por cáncer metastásico. Es una manifestación tardía que implica mal pronóstico. El diagnóstico puede ser difícil y requiere de un alto índice de sospecha. La embolia pulmonar tumoral es la presencia de múltiples acúmulos de células tumorales en las pequeñas arterias, arteriolas y capilares septales del pulmón. La oclusión de la microvasculatura pulmonar por células tumorales y trombos asociados puede producir una entidad clínica subaguda y progresiva que se asemeja a la enfermedad tromboembólica . Algunas modalidades de imágenes pueden ayudar a definir el diagnóstico, especialmente gammagrafía pulmonar de ventilación-perfusión y su característico patrón de múltiples defectos de perfusión sub- segmentarios periféricos sin alteraciones ventilatorias o en la angiografía. El tratamiento no ha sido ampliamente estudiado, ya que el diagnóstico por lo general no se realiza hasta después de la muerte. Se realiza una revisión de la literatura sobre la evaluación y el diagnóstico de estas dos entidades clínicas poco frecuentes.

Lymphangitic carcinomatosis is a relatively uncommon disorder characterized by diffuse infiltration of the pulmonary lymphatics by metastatic cancer. It is a late manifestation of malignancy that implies bad prognosis. The diagnosis can be difficult and requires a high degree of suspicion. Microscopic pulmonary tumor embolism is the presence of multiple aggregates of tumor cells in the small pulmonary arteries, arterioles and septal capillaries. Occlusion of the pulmonary microvasculature by tumor cells and associated thrombi can produce a subacute and progressive clinical entity that resembles thromboembolic disease. Some imaging studies may help to define the diagnosis, especially the ventilation-perfusion lung scan and its typical pattern of multiple peripheral sub-segmental perfusion defects without ventilatory abnormalities or the angiography. Treatment for this entity has not been extensively studied, since the diagnosis is usually made postmortem. We provide a review of the literature about the evaluation and diagnosis of these two infrequent clinical entities.

Linfangitis carcinomatosa pulmonar: presentación como neumopatía aguda. Reporte de dos casos / [Pulmonary carcinomatous lymphangitis: presentation as acute pneumopathy. Report of two cases].

INTRODUCTION: Carcinomatous lymphangitis of the lung accounts for 6-8

of lung’s metastases. There are evidence that it can be a treatable condition with an impact on progression of dyspnea and radiographic lesions, and survival improvement. Two cases are reported, with the aim of increase the clinical suspicion at compatible cases. METHODS: Case 1: woman 32 years old. Progressive dyspnea and cough two weeks ago, without antibiotic response. Tachypnea, increase of respiratory work, basal crackles. Respiratory failure. Radiography: basal alveolointerstitial opacities. Treatment for severe community-acquired pneumonia is started. She evolves unfavorably, with need of MRA and fatal outcome. Case 2: woman 46 years old. Progressive dyspnea and cough from one week ago. Tachypnea, diffuse crackles. Respiratory failure. Radiography: diffuse nodular-interstitialradiopacity, with radiopacy lesion in right apex. HRCT: nodular thickening of interlobular septum and peribrochovascular interstitial. Treatment for tuberculosis of the lung is started. She evolves unfavorably, with need of MRA and fatal outcome. In both cases a lung biopsy was performed, diagnosing carcinomatous lymphangitis. DISCUSSION: Two cases are reported, with acute afebrilepneumopathyin young patients, whose manifestations guided to treatment of respiratory infection, and evolved quickly to refractory hypoxemic respiratory failure with need of MRA and fatal outcome. CONCLUSION: Carcinomatous lymphangitis of the lung should be included as a differential diagnosis of cases of acute lung pathology, especially when there was an unfavorable evolution under antibiotics or have excluded more common etiologies.

Primary Cutaneous Nocardiosis with Sporotrichoid Pattern by N. brasiliensis in Lung Cancer Patient / 대한피부과학회지

Nocardia is aerobic gram positive bacteria, which is found in the soil. Cutaneous nocardiosis is divided into the primary skin infection and secondary infection due to systemic dissemination from the internal organ. Its clinical manifestations are mycetoma, lymphocutaneous infection and superficial infection, such as abscess or granuloma. In lymphocutaneous infection, it is important to make differential diagnosis with other diseases with sporotrichoid pattern, such as sporotrichosis, leishmaniasis and mycobacterial infection. In our case, 58 year old male who had history of chemotherapy due to lung cancer visited with granulomatous nodule on the left wrist, and following lymphangitis along the forearm for 2 months. Through a skin biopsy, culture, AFB stain and 16S rRNA gene sequencing, cutaneous nocardiosis by N. brasiliensis was diagnosed.

Intralymphatic accumulation of lymphocytes mimicking intravascular lymphomatosis / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the significance and differential diagnosis of intralymphatic accumulation of lymphocytes.</p><p><b>METHODS</b>The clinical and pathologic features of 4 cases of intralymphatic accumulation of lymphocytes were reviewed retrospectively. Immunohistochemical study was carried out and follow-up data were analyzed.</p><p><b>RESULTS</b>The sites of involvement included tonsil (2 cases), pharynx (1 case) and appendix (1 case). The duration of disease ranged from 1 week to 3 months. Follow up of the patients (from 3 to 84 months) showed no evidence of disease recurrence. Gross examination of the tissues (except in the case of appendiceal involvement) showed polypoid changes. Histologically, the lymphatic channels were filled up with small lymphocytes and associated with fibrosis in the vicinity. Immunohistochemical study revealed a T-cell phenotype of the intralymphatic lymphoid cells.</p><p><b>CONCLUSIONS</b>The accumulation of lymphocytes in lymphatic channels is associated with a benign clinical course. This phenomenon may be due to retention of lymphocytes secondary to the perilymphatic chronic inflammation and fibrosis. Although the lesion simulates intravascular lymphomatosis morphologically and shows a uniform T-cell phenotype, the lymphoid cells lack obvious cellular pleomorphism and mitotic activity. The solitary nature of the lesion, when coupled with the indolent clinical behavior, is also helpful in the differential diagnosis.</p>

Surgical vacuum-assisted closure for treatment of dramatic case of stonefish envenomation / 中华创伤杂志(英文版)

Skin necrosis of the foot, oedema and lymphangitis from stonefish (Synanceia verrucosa) sting are complications well known for a long time and with potential long-term sequelae. Literature reports of stonefish envenomation give no specific reference on soft tissue management and surgical reconstruction. This is the first report describing a case of foot stonefish envenomation treated by vacuum-assisted closure therapy as an easy to use, accessible and simple adjuvant tool for management of large soft tissue necrosis.

Elefantiasis: a razón de un caso / Elephantiasis: apropos of a case

Se presenta un caso de una paciente de 80 años operada por cáncer de vulva, a la que se le realizó vulvectomía radical con vaciamiento inguinal bilateral hace 20 años, que como secuela presenta elefantiasis bilateral. A propósito, se realiza revisión de la literatura planteando algunas consideraciones generales.

We present the case o fan 80-years-old woman operated of vulva cancer, who was the subject of a radical vulvectomy with bilateral inguinal draining 20 years ago, presenting bilateral elephantiasis as a sequel. Apropos of this case, we reviewed the literature and stated some conclusions.

Linfangite granulomatosa de genitália infantil: relato de caso e revisão da literatura / Granulomatous lymphangitis of children's genitalia: report of a case and review of the literature

Linfangite granulomatosa de genitália não-infecciosa é causa rara de linfedema genital autolimitado e idiopático em crianças(4, 6, 9). A maioria dos casos de linfedema com granuloma não-infeccioso de genitália em pacientes jovens ocorre em associação à doença de Crohn subseqüente ou concomitante(1, 3, 4, 9). O caso relatado é de um menino de 14 anos que apresentava história de linfedema genital principalmente em região dorsal do corpo do pênis, poupando prepúcio, parte distal do pênis e saco escrotal. Estudos laboratoriais não revelavam doenças sistêmicas. O exame anatomopatológico da peça revelou tratar-se de linfangite granulomatosa não-infecciosa.

Non-infectious granulomatous lymphangitis of genitalia is a rare cause of self-limited and idiopathic genital lymphoedema in children(4, 6, 9). Most cases of lymphoedema with non-infectious genital granulomas in young patients occur subsequent to or concomitant with Crohn's disease(1, 3, 4, 9). The case described is of a 14-year-old boy with history of genital lymphoedema mainly on the dorsal region of the body of the penis, sparing the prepuce, the distal part of the penis and the scrotum. Laboratory studies did not reveal systemic diseases. The histopathological analysis showed it was non-infectious granulomatous lymphangitis.

Linfangite carcinomatosa: impacto da terapêutica paliativa na qualidade de vida e prognóstico / Neoplastic pulmonary lymphangitis (NPL) is the intrapulmonary spread of metastatic neoplasm via lymphatic tissue, and accounts for 6-8% of lung metastasis: symptom distress has great impact on quality of life

A linfangite pulmonar neoplásica (LPN) é a disseminação intrapulmonar de células neoplásicas metastáticas via vasos linfáticos, englobando 6 a 8% das metástases pulmonares. Seu desconforto sintomático tem grande impacto na qualidade de vida (QdV) e seu prognóstico é muito reservado. Nosso objetivo foi avaliar os aspectos clínicos, o tratamento, a QdV e a sobrevida em LPN, em um estudo prospectivo. O seguimento mediano foi de 32 dias; 79% dos pacientes foram seguidos por menos de 6 meses, devido a evolução desfavorável e óbito. A idade mediana dos pacientes foi de 60,5 anos; 65% eram mulheres. A maioria dos sítios primários de neoplasia foram pulmão e mama (88%). A QdV estava comprometida, mas podiam ter algum benefício terapêutico, especialmente com corticosteróides, e a quimioterapia melhorou a sobrevida. Algumas variáveis foram estatisticamente significantes para predizer o status de sobrevida em um mês: Karnofsky performance scale (KPS)>=60%, hemoglobina(Hb)>=10 g/dL, LPN unilateral, PO2>=60 mmHg, tratamento oncológico sistêmico e sem necessidade de ventilação assistida não-invasiva. Preditores significantes de maior sobrevida mediana foram: KPS>=60%, SpO2>=90%, Hb>=10 g/dL, sem hipoxemia (PO2>=60 mmHg), sem necessidade de oxigenioterapia, tratamento com opióide fraco, tratamento oncológico sistêmico e ausência de ventilação assistida. O tratamento sintomático deve ser ativo, oferecendo melhor QdV aos pacientes e familiares e o tratamento etiológico é importante. As avaliações de QdV facilitam a relação médico-paciente, além da informação prognostica ser crucial para ajudar na definição e planejamento terapêuticos, uma vez que análises de parâmetros de custo-benefício e metas realistas são essenciais para cuidados oncológicos paliativos de boa qualidade, especialmente em síndromes com prognóstico reservado, tal como a LPN.

Our objective was to evaluate clinical aspects, treatment, QoL, and survival, in NPL in a prospective study. Median follow-up was of 32 days; 79% percent of patients were followed for less than 6 months, due to bad outcome. Median age was 60.5 years; 65% were female. Most primary tumor sites were lung and breast cancers (88%). QoL was compromised, but could have treatment benefit, especially with corticosteroids, and chemotherapy improved survival. Some variables were statistically significant for predicting survival status in one month, such as: Karnofsky performance scale (KPS)>=60%, hemoglobin>=10 g/dL, unilateral NPL, PO2>=60 mmHg, oncological systemic treatment, and no need for noninvasive assisted ventilation. Significant predictors of longer median survival were: KPS>=60%, SpO2>=90%, hemoglobin>=10 g/dL, without hypoxemia (PO2>=60 mmHg), no need for oxygen therapy, weak opioid treatment, systemic oncological treatment, and absence of assisted ventilation. Symptomatic treatment must be active, offering better QoL to patients and their families, and etiological treatment is important. QoL evaluation facilitates patient-physician relationship, and prognostic information is crucial to help in defining treatment plan, since cost-benefit parameter analysis and realistic goals are essential in good-quality palliative cancer care, especially for syndromes with poor prognosis, such as NPL.