Kaposi sarcoma following postmastectomy lymphedema

Classical Kaposi sarcoma (KS) usually appears on lower extremities accompanied or preceded by local lymphedema. However, the development in areas of chronic lymphedema of the arms following mastectomy, mimicking a Stewart–Treves syndrome, has rarely been described. We report an 81‐year‐old woman who developed multiple, erythematous to purple tumors, located on areas of post mastectomy lymphedema. Histopathological examination evidenced several dermal nodules formed by spindle‐shaped cells that delimitated slit‐like vascular spaces with some red cell extravasation. Immunohistochemically, the human herpesvirus type 8 (HHV‐8) latent nuclear antigen‐1 was detected in the nuclei of most tumoral cells confirming the diagnosis of KS. Lymphedema could promote the development of certain tumors by altering immunocompetence. Although angiosarcoma (AS) is the most frequent neoplasia arising in the setting of chronic lymphedema, other tumors such as benign lymphangiomatous papules (BLAP) or KS can also develop in lymphedematous limbs. It is important to establish the difference between AS and KS because their prognosis and treatment are very different. Identification by immunohistochemistry of HHV‐8 is useful for the distinction between KS and AS or BLAP.     

从“血不利则为水”探讨乳腺癌相关淋巴水肿

导致血不利的原因一是不足,即由于气、血、津、液等物质的缺乏致无源行血;一是不通,即水、湿、痰、饮、瘀、食积、火郁、内风、外伤等病理因素阻滞,致血行不畅。血瘀经脉之内,则水亦瘀积脉中,致脉络胀满,形成水肿。乳腺癌术后患者,金刃本已损伤血脉,术后的放疗、化疗又属祛邪之法,故机体元气受损,气虚无力行血,血运行不畅,导致患侧上肢水肿。故治疗乳腺癌相关淋巴水肿时,应当血水同治,即活血利水之法要贯穿始终。先病血而后病水者,可以活血化瘀为主,利水为辅;先病水后病血者,则以利水消肿为主,酌加活血养血之品。但需要注意的是:①临证时切不可拘泥于单纯的活血利水法,而忽视乳腺癌相关淋巴水肿(breast cancer related lymphedema,BCRL)患者机体本身的状况。②临证勿忽视五脏与血、水的关系,以及肺、脾、肾、三焦与水肿的内在关系。③可多种方法联合运用,审证求因,标本同治。BCRL术后宜补气活血,通脉利水;术后兼化疗者可疏肝健脾、利水消肿;早期可在活血利水基础上,侧重利湿消肿,后期多湿聚为痰,治疗侧重化痰软坚。     

Lymphangiosarcoma in late-onset hereditary lymphedema: Case report and nosological implications

Hereditary lymphedemas that are not associated with other malformations usually affect the lower limbs and are inherited in an autosomal dominant fashion. These non-syndromic hereditary lymphedemas are categorized by their age of onset, being either congenital (Milroy disease) or having an onset in childhood or around puberty (Meige disease). We describe a family in which three individuals in three generations had unusually late onset of lym-phedema in their mid-twenties or thirties. The proband additionally developed a very rare lymphangiosarcoma. This tumor, usually associated with post-mastectomy lym-phedema, has not been described in late-onset hereditary lymphedema. Because of an unusually high incidence of multiple primary tumors in association with lymphangiosarcoma in the literature (approximately 10%) and the proband's own familial cancer background, we speculate that an inherited predisposition to malignancy may underlie the development of lymphedema-associated lymphangiosarcoma. © 1995 Wiley-Liss, Inc.     

Chy-3 mice are Vegfc haploinsufficient and exhibit defective dermal superficial to deep lymphatic transition and dermal lymphatic hypoplasia.

Recent advances in molecular lymphology and lymphatic phenotyping techniques in small animals offer new opportunities to delineate mutant mouse models. Chy-3 mutant mice were originally named for their chylous ascites, but the underlying lymphatic disorder was not defined. We now re-examined these mice and applied advanced genotyping and lymphatic phenotyping techniques to pinpoint the specific lymphatic defect in this mouse model. We demonstrated that Chy-3 mice carry a large chromosomal deletion that includes Vegfc and narrowed this region by monitoring the heterozygosity of genetic markers. We found that Chy-3 mice not only exhibited chylous ascites but also lymphedema of the hind paws and, in approximately half of the males, lymphedema of the penis. Visual lymphangiography and immunofluorescence staining showed a hypoplastic dermal lymphatic network, whereas the blood vasculature appeared unaffected. This hypoplastic lymphatic network was functional, and all adult Chy-3 mice exhibited a lateral lymphatic pathway directly connecting the inguinal to the axillary lymph node. The dermal superficial to deep lymphatic connections in upper limbs and in all cervical regions were intact and functionally drained the upper body. Lymphatic tracer was not transported from the dermal to the deep truncal lymphatic system in the lower limbs, even though the deep lymphatic vessels and nodes were present and patent. These findings further delineate the lymphatic phenotype of Chy-3 mice, identify a collateral lymph drainage pathway previously undescribed in other genetic models of lymphedema, and demonstrate a predilection for lymphatic abnormalities of the lower limbs.     

Pericentric inversions of chromosome 4: report of a new family and review of the literature

A family was cytogenetically studied because of the birth of a male child with a multiple congenital anomaly pattern, in whom a dup (4q) recombinant was found. His phenotypically normal mother's karyotype showed an apparently balanced pericentric inversion in a chromosome 4. So as to analyze the occurrence of recombinants, the cytogenetic data from this family are compared with those of the 18 previously reported familial cases of pericentric inversions (PIs) of chromosome 4. The congenital anomalies observed in the child strongly suggest Wolf-Hirschhorn syndrome but some of his clinical features seem to be pathogenetically related to the presence of lymphedema during the intrauterine period. In the multiple congenital anomaly pattern observed in this patient, the lymphedema could be the consequence of the large 4q duplication. The review of chromosome 4 PIs with 4q duplication suggests that the q3 region should be examined when edema is detected prenatally.     

Dominantly inherited syndrome of microcephaly and congenital lymphedema

A Chinese family is reported in which microcephaly and congenital lymphedema have been observed in at least 4 generations. This combination of symptoms can be presumed to represent a rare but well defined hereditary syndrome transmitted by an autosomal dominant gene.     

DSA引导下腰交感神经阻滞治疗妇科肿瘤术后下肢淋巴水肿

目的观察DSA引导下腰交感神经阻滞治疗妇科肿瘤术后下肢淋巴水肿的有效性及安全性。方法回顾性分析20例妇科肿瘤术后下肢淋巴水肿患者,均接受DSA引导下腰交感神经阻滞治疗(均治疗2次)。分别于阻滞前、首次阻滞后第1天及第2次阻滞后第1、7天测量患侧腿围,记录患侧腿围缩小值。采用Inbody720多频生物电阻人体成分分析仪检测阻滞前及第2次阻滞后1周时的组织水肿程度;观察症状改善及功能恢复情况。结果 20例均顺利完成阻滞。1例术后出现短暂下肢无力,余无不良反应。首次阻滞后第1天及第2次阻滞后第1、7天患侧下肢各测量点腿围均不同程度缩小,且各测量点第2次阻滞术后第1、7天腿围缩小值均高于第1次术后第1天(P均0.01)。第2次阻滞后1周组织水肿程度较术前降低(P0.01),髋关节活动度较术前增加(P0.01),患侧下肢肿胀感、紧绷感、疼痛感、麻木感均消失。结论 DSA引导下腰交感神经阻滞治疗妇科肿瘤术后下肢淋巴水肿有效且安全。     

EFFECTS OF MICROWAVE TREATMENT ON THE LYMPHOCYTESUBSETS IN CASES OF CHRONIC LYMPHEDEMA OF EXTREMITIES

R68llm6 Objectif Pour determiner l'dtat immunitaire des sons-groups l~taires T des malades aveclwtoeddlne chronique des extremitys et l' effet du traitement per micrreondes. ANt~ ie malades avec Iy7nphedimedes extradites(n = 20 ) et ies volontaires no~ux (n = 10 ) out ate studies per FITC on PHmerque MOAbs et cytometrie dcouleur double pour erminer ies changements des Phenotype l~taires T chez ies malades avec lpephoeddnZe aunt et aprdstraitement. ~flats Le rdsultat a montrd que ie pourc…     

Long-term results in the surgical treatment of lymphedema

Summary In the irreversible stage of lymphedema corrective surgery is often mandatory. A choice has to be made between two different basic selective procedures. As no totally satisfactory surgical method has as yet been established surgical management is difficult and implies considerable personal responsibility. This is a report on the long-term results of operations carried out on 16 patients between 1958 and 1979 using the so called Charles procedure. It concludes that this radical operation gives an acceptable long-term result in long-standing, elephantiastic primary lymphedema of the lower extremity.