RESUMEN

OBJECTIVES: Angiosarcomas are rare malignant mesenchymal neoplasms of endothelial cell origin with a predilection to the ventral abdominal wall in cats. Larger case series describing this entity are lacking. METHODS: Two referral centre laboratory databases were searched for angiosarcoma of the ventral abdominal wall. Nine cases with a histological diagnosis were included. Immunohistochemistry (factor VIII and PROX-1 antibodies) was used to phenotype them as haemangiosarcoma or lymphangiosarcoma. RESULTS: All cats presented with a ventral abdominal mass, five of which were producing a serosanguinous discharge. Eight underwent tumour staging and pulmonary metastases were suspected in one cat (but not histologically confirmed). With histopathology alone, a diagnosis of angiosarcoma and lymphangiosarcoma was made in four and five cases, respectively. After immunohistochemistry, five cases had a haemangiosarcoma phenotype and four had a lymphangiosarcoma phenotype, including two cases of lymphangiosarcoma that were reclassified as hemangiosarcoma. Eight cats received treatment (either surgery with or without adjuvant therapies or medical management alone). Six cats were euthanased due to local disease progression. The median survival time for haemangiosarcoma was 166 days (range 137-381), and for lymphangiosarcoma it was 197 days (range 67-208). Two cats with haemangiosarcoma remained alive for a follow-up period of 329 and 580 days, respectively. CONCLUSIONS AND RELEVANCE: Feline ventral abdominal angiosarcomas are rare locally aggressive neoplasms. While histology often provides a diagnosis of angiosarcoma, immunohistochemistry is ultimately required to differentiate between haemangiosarcoma and lymphangiosarcoma phenotypes. Further studies are required to evaluate whether the different phenotypes have an impact on treatment response and outcome.

Asunto(s)

RESUMEN

A 3-year-old Great Dane presented with a history of chronic vomiting and diarrhoea. Abdominal computed tomography followed by exploratory laparotomy revealed a perforated, segmental partial mesenteric volvulus, affecting an abnormal section of distal jejunum, which was resected. Histopathology and immunohistochemistry results were consistent with jejunal lymphangiosarcoma. This case represents the first report of primary small intestinal lymphangiosarcoma in dogs and the importance of immunohistochemistry for definitive diagnosis.

Asunto(s)

RESUMEN

Abstract Background stewart-treves syndrome (STS) is an angiosarcoma associated with chronic lymphedema. Objectives This article analyses the characteristics of twenty-two patients and proposes active intervention in lymphedema and the early diagnosis of STS. Methods Twenty-two patients with STS were diagnosed at the centre over an 11-year period. Clinical manifestations, a series of conventional analyses, and histopathology were used to study these cases retrospectively. Results The age range of 22 patients with STS was 15 to 78 years. The main clinical manifestations included multiple skin and subcutaneous nodules and scattered red or purplish-red rashes in the lymphoedematous limbs. These patients often showed clinical symptoms such as lymphedema, weakness, emaciation, pain, mass, lymphadenopathy and so on. The positive rates of ultrasonography, MRI and radionuclide imaging were 66.7% (6/9), 92.3% (12/13) and 18.2% (2/11), respectively. The main points regarding active intervention in lymphedema and early diagnosis of STS were summarized. Study limitations Since this was a retrospective study, the main points summarized by the author need to be further quantified in clinical work to guide the diagnosis of this kind of disease more conveniently. In addition, further clinical trials are needed to evaluate the role of lymphedema in the occurrence and development of malignant tumors. Conclusions STS can appear in lymphoedematous tissue many years after lymphedema onset. To avoid delays in the diagnosis and therapy of STS, physicians should actively look for signs or symptoms of malignant lymphedema during the follow-up period and promptly manage patients developing problems.

RESUMEN

Lymphatic malformation (LM) is a vascular anomaly from lymphatic endothelial cells (ECs), and a fraction of the patients could progress to the deadly malignant lymphangiosarcoma (LAS). Using genetic tools to delete an essential autophagy gene Rb1cc1/FIP200 or its mutation specifically blocking its autophagy function, we demonstrated that autophagy inhibition abrogated LM progression to LAS although not affecting LM formation in our recently developed mouse model of LAS. Analysis of the mouse models in vivo and vascular tumor cells in vitro showed that autophagy inhibition reduced vascular tumor cell proliferation in vitro and tumorigenicity in vivo without affecting mTORC1 signaling as an oncogenic driver directly. Transcriptional profiling of autophagy-deficient tumor cells and further mechanistic studies revealed a role for osteopontin (OPN) and its downstream Jak/Stat3 signaling in mediating regulation of vascular tumor cells by autophagy. Together, these results support potential new prophylactic strategies to targeting autophagy and/or its downstream OPN expression to prevent progression of the benign LM to the malignant and deadly LAS.Abbreviations: LM: lymphatic malformation; EC: endothelial cell; LAS: lymphangiosarcoma; OPN: osteopontin; RB1CC1: RB1 Inducible Coiled-Coil 1; FIP200: FAK family-interacting protein of 200 kDa.

RESUMEN

BACKGROUND: stewart-treves syndrome (STS) is an angiosarcoma associated with chronic lymphedema. OBJECTIVES: This article analyses the characteristics of twenty-two patients and proposes active intervention in lymphedema and the early diagnosis of STS. METHODS: Twenty-two patients with STS were diagnosed at the centre over an 11-year period. Clinical manifestations, a series of conventional analyses, and histopathology were used to study these cases retrospectively. RESULTS: The age range of 22 patients with STS was 15 to 78 years. The main clinical manifestations included multiple skin and subcutaneous nodules and scattered red or purplish-red rashes in the lymphoedematous limbs. These patients often showed clinical symptoms such as lymphedema, weakness, emaciation, pain, mass, lymphadenopathy and so on. The positive rates of ultrasonography, MRI and radionuclide imaging were 66.7% (6/9), 92.3% (12/13) and 18.2% (2/11), respectively. The main points regarding active intervention in lymphedema and early diagnosis of STS were summarized. STUDY LIMITATIONS: Since this was a retrospective study, the main points summarized by the author need to be further quantified in clinical work to guide the diagnosis of this kind of disease more conveniently. In addition, further clinical trials are needed to evaluate the role of lymphedema in the occurrence and development of malignant tumors. CONCLUSIONS: STS can appear in lymphoedematous tissue many years after lymphedema onset. To avoid delays in the diagnosis and therapy of STS, physicians should actively look for signs or symptoms of malignant lymphedema during the follow-up period and promptly manage patients developing problems.

Asunto(s)

RESUMEN

Background: Stewart-Treves syndrome (STS) is a lymphatic sarcoma secondary to chronic lymphedema of the extremities. Most STS patients have a history of breast cancer and have undergone radical mastectomy and postoperative radiation and chemotherapy. It usually occurs 11 to 12 years after surgery, and about 0.45% of patients are estimated to have the disease. The characteristics of STS include that it is clinically relatively rare, has a high degree of malignancy, can spread easily in the absence of timely treatment, and has low survival rate. Herein, we report a case of STS which developed 13 years after breast cancer-related lymphoedema (BCRL). It allows doctors to recognize and detect the disease earlier. Case Description: A 74-year-old woman had undergone modified radical mastectomy 13 years ago for invasive ductal breast cancer in her left breast. After multiple rounds of postoperative chemoradiotherapy, multiple purple lesions were found in the left upper limb during physical examination in April 2021. The lesions spread rapidly and were varied in size. An immediate skin biopsy reported the lesions as STS. The patient was diagnosed with lymphangiosarcoma with metastasis (STS). The surgical method was shoulder joint amputation, chest wall resection, and local flap transfer. After surgery, the patient underwent 6 rounds of paclitaxel 300 mg + carboplatin 300 mg chemotherapy. After chemotherapy, the patient's wound healed and the suspected metastasis disappeared. At the time of writing, she has survived for more than 13 months, and her quality of life has improved significantly, to the satisfaction of the patient and her family. The patient is able to eat normally and lead a normal life with some assistance, without significant weight loss. Conclusions: Although rare, STS is a serious invasive complication of breast cancer surgery. To increase their relative survival time, patients with BCRL need to identify and thoroughly investigate rapidly progressing skin lesions, and undergo timely surgery.

RESUMEN

Cystic lymphangioma (CL) is a rare benign tumor that mainly occurs in the neck and axilla and usually occurs in children, whereas lymphangiosarcoma (LAS) is a rare invasive tumor, usually secondary to chronic lymphedema caused by various causes, with a rare malignant transformation from CL. We presented the case of a 63-year-old woman who underwent four surgical excisions for multiple recurrence of CL in the right groin. The changes of imaging and pathological examination revealed the unusual process of its gradual malignant transformation into LAS. We followed up the patient for 16 years, and she eventually died of LAS complications.

RESUMEN

A Síndrome de Stewart-Treves é uma rara condição onde um angiossarcoma se desenvolve sobre área de linfedema crônico. Afeta mais frequentemente os membros superiores e representa 5% dos casos de angiossarcoma. Apesar de ser mais comum em mulheres submetidas a mastectomia radical e esvaziamento axilar por neoplasia de mama, pode ocorrer a partir de linfedema de outras etiologias. Clinicamente as lesões são caracterizadas por nódulos únicos ou múltiplos de cor avermelhada ou azulada no estágio inicial e, em estágio avançado, podem evoluir para massas hemorrágicas e até gangrena. O prognóstico é ruim e a conduta cirúrgica com excisão ampla ou amputação oferecem uma maior chance de sobrevida. Relata-se um caso de Síndrome de Stewart-Treves na perna direita, apresentando-se de forma exuberante e com acometimento de linfonodos pélvicos e abdominais.

Stewart-Treves syndrome is a rare condition characterized by the development of an angiosarcoma at sites of chronic lymphedema. It most often affects the upper limbs and accounts for 5% of cases of angiosarcoma. Although the syndrome is more common in women undergoing radical mastectomy and axillary dissection for breast cancer, it may result from lymphedema of other etiologies. Clinically, the lesions are characterized by single or multiple bluish-red nodules in the early stage and may progress to hemorrhagic masses and even gangrene in advanced stages. The prognosis is poor, and surgical management with wide excision or amputation offers a greater chance of survival. We report a case of profuse Stewart-Treves syndrome in the right leg, with involvement of pelvic and abdominal lymph nodes.

Asunto(s)

RESUMEN

Background: Angiosarcomas are a broad category of vascular origin neoplasms that are poorly characterized in veterinary species. Lymphangiosarcoma (LAS) is an uncommon type of angiosarcoma reported in humans and canines arising from lymphatic endothelium. LAS can be differentiated from other angiosarcomas in dogs based on expression of Prospero-related homeobox gene-1 (PROX-1) or lymphatic vessel endothelial receptor-1 (LYVE-1). Composite hemangioendothelioma (CHE) is a rare angiosarcoma subtype described in people and characterized by a variable biologic behavior and infrequent metastasis. This variant of angiosarcoma histologically combines features of retiform hemangioendothelioma and epithelioid hemangioendothelioma. Information regarding the cytologic and histopathologic appearance and clinical course of dogs with vascular tumors that exhibit features of CHE are unknown. Here, we report a case of pleomorphic LAS with features of CHE arising in a dog and treated with surgery and adjuvant chemotherapy. Case presentation: A 10-year-old intact male Labrador retriever presented with an approximately 6-cm-diameter cutaneous mass caudal to the left elbow that was progressively growing over 1.5 years. On physical examination, palpable extensions were identified coursing proximally over the triceps with concurrent loco-regional peripheral lymphadenopathy. Fine needle aspirates (FNA) and cytologic assessment of the cutaneous mass, left prescapular, and accessory axillary lymph nodes reported that this appeared to be a metastatic epithelial neoplasm, although a mixed carcinoma or collision tumor could not be excluded. An incisional biopsy of the mass was submitted for histopathology and was consistent with a well-differentiated angiosarcoma with features of CHE. The neoplasm expressed vimentin, CD31, von Willebrand factor (vWf), and PROX-1, supporting the diagnosis of LAS. Complete staging was performed, and no additional metastatic lesions were identified. Left forelimb amputation and lymph node removal were performed. Based on the diagnosis of metastatic LAS, doxorubicin chemotherapy was administered. 7 months post-amputation, the tumor recurred at the amputation site without evidence of metastatic disease. Conclusion: This report describes a malignant, locally aggressive lymphatic origin vascular tumor in a dog, with features consistent with descriptions of CHE in humans. Cytologic features in this case were discordant with its true mesenchymal etiology, obfuscating diagnosis. The morphologic features of the mesenchymal neoplastic population and immunohistochemistry (IHC) labeling ultimately supported a diagnosis of pleomorphic LAS with features of CHE.

RESUMEN

Stewart-Treves syndrome (STS) is an angiosarcoma that usually develop in an extremity with longstanding lymphedema. Most affected patients have a history of breast cancer treated with radical mastectomy. Here, we report a case of STS with breast cancer-related lymphedema (BCRL) for a period of seven years. A 56-year-old woman presented with chronic lymphedema of the left arm. Nine years previously she had modified radical mastectomy for grade 2, invasive, ductal breast cancer. Upon physical examination, a tender, purplish lesion on the medial half of the affected arm was observed. The lesion spread rapidly with different-sized, scattered, purple-colored lesions in the affected area. A prompt skin biopsy was reported as STS. An immediate arm amputation was performed. However, a few months later she presented with new lesions on the anterior thorax and subsequent local recurrence around the scar. She received radiation-therapy. However, six months later the angiosarcoma had spread to the pelvic and upper limb area with scattered skin lesions. She had several problems during the chemotherapy and radiation-therapy, although she survived beyond 20 months. In conclusion, STS is a rare but aggressive and important complication of BCRL. Awareness of rapidly progressing skin lesions and detailed investigation, as well as prompt surgery is necessary for patients with BCRL in order to relatively increase the survival time.

RESUMEN

Détention d'armes et permis de chasser. Les armes à feu sont la cause de plus d'un millier de décès chaque année, représentant ainsi un objectif de santé publique conséquent. Pour les armes à feu de type B et C, le tir sportif et le permis de chasse, le médecin traitant joue un rôle crucial, délivrant à chaque fois un certificat selon des modalités bien particulières.

Asunto(s)

RESUMEN

Lymphangiosarcoma, or Stewart-Treves Syndrome (STS), is a very rare skin angiosarcoma with poor prognosis, which usually affects the upper limbs of patients who underwent breast cancer surgery, including axillary dissection followed by radiotherapy (RT). Cutaneous lymphangiosarcomas, which account for approximately 5% of all angiosarcomas, usually originate in the limb with chronic lymphedema. Lymphatic blockade is involved in the onset of STS. RT contributes indirectly to an increased risk of developing STS by causing axillary-node sclerosis and resulting in a lymphatic blockade and lymphedema. Chronic lymphedema causes local immunodeficiency, which indirectly leads to oncogenesis. Currently, axillary nodes are no longer routinely irradiated after axillary dissection, which is associated with a reduction in the incidence of chronic lymphedema from 40% to 4%. The use of sentinel lymph node biopsy technique is also widespread and the associated risk of lymphedema is further reduced. Thus, the incidence of STS decreased significantly with improved surgical and radiation techniques. The overall prognosis of STS patients is very poor. Only early radical surgical removal, including amputation or disarticulation of the affected limb, or wide excision at an early stage offers the greatest chance of long-term survival. Only a few case reports and series with a small number of patients with lymphangiosarcoma can be found in the literature. We present a case report of the first diagnosed STS at our department in an effort to highlight the need of the consideration of developing lymphangiosarcoma in patients with chronic lymphedema.

RESUMEN

Abdominal ultrasonographical and computed tomography examinations of a 12-year-old neutered female toy poodle revealed a protruding mass, approximately 2 cm in diameter, at the apex of the bladder. The mass was firm and haemorrhagic with a homogeneously brownish-yellow cut surface. Microscopically, it was unencapsulated and located in the muscle layer with invasion of the extra-muscular layer. It was composed of spindloid to oval neoplastic cells that formed irregular clefts and diffuse sheets that dissected bundles of collagen. Immunohistochemically, the neoplastic cells were positive for vimentin and lymphatic vessel endothelial hyaluronan receptor 1 antigens, but negative for cytokeratin AE1/AE3, factor VIII-related antigen, CD31, CD34, Prox-1, S100, desmin, α-smooth muscle actin and MyoD1. Negative immunolabelling for laminin antigen supported the absence of evidence of a basal lamina on ultrastructural examination. Based on these findings, this tumour was identified as a lymphangiosarcoma. To the best of our knowledge, this case is the first report of lymphangiosarcoma arising from the bladder in a dog.

Asunto(s)

RESUMEN

An 11-y-old spayed female German Shepherd was presented for a second opinion of ventral cervical swelling of 3-mo duration. On examination, the dog had significant dependent ventral cervical swelling. Enlarged lymph nodes with cystic changes and severe edematous facial swelling were noted on computed tomography. Fine-needle aspiration of the ventral cervical swelling revealed yellow-tinged fluid, with a predominance of lymphoid cells noted on cytologic examination. On cervical exploratory surgery, the left mandibular lymph node was surrounded by a large fluid pocket; biopsies of the lymph node were obtained. Impression smear cytology, flow cytometry, PCR for antigen receptor gene rearrangements, and histopathology were performed on samples from the left mandibular lymph node. Impression smear cytology revealed a population of atypical discrete cells. Flow cytometry identified a population of CD34+/CD45- large cells. A tumor of endothelial origin within the medulla of the lymph node was identified by histopathology, and lymphangiosarcoma was confirmed based on prospero-related homeobox gene 1 (PROX1) immunoreactivity. Our study describes the challenges in the diagnosis of a rarely reported entity and highlights that neoplastic endothelial cells should be considered as a differential when high proportions of CD34+/CD45- cells are present in flow cytometry.

Asunto(s)

RESUMEN

AIMS: To demonstrate clinicopathologic features of Stewart-Treves syndrome (STS) including clinical manifestations, morphology, immunophenotype (especially c-MYC amplification), differential diagnosis, pathogenesis, treatment and prognosis. METHODS AND RESULTS: 17 cases of STS were retrospectively archived, involving 6 cases of postmastectomy, 3 cases of postoperative cervical cancer and 8 cases of chronic lymphatic obstruction without history of malignancy. Seven of 9 cancer patients had undergone radiotherapy. All the patients presented with lymphedema as the first sign. The lesions appeared as multiple reddish blue macules or nodules with polypoid and coalesce. Microscopic examination revealed infiltrative proliferation of irregular vessels in dermis and subcutaneous tissue. The tumorous endothelial cells displayed pleomorphism in morphology. The heteromorphic tumor cells expressed CD34, CD31, ERG, D2-40, c-MYC and factor VIII. Despite various treatment modalities, all cases died in an average of 13.6 months, with 1 case of loss to follow-up. CONCLUSIONS: STS is an extremely rare malignancy that arises from congenital or secondary chronic lymphedema. STS uniquely overexpressed c-MYC. In spite of poor prognosis, early detection is important to facilitate a full range of available therapies, even an opportunity for curative treatment. A low threshold for biopsy and early referral to an experienced multidisciplinary team are highly recommended for optimum management.

RESUMEN

We herein report the case of a 3-year-old girl with atypical congenital right upper limb lymphedema who developed an angiosarcoma. Only a few cases have been reported following congenital form of lymphedema and only 4 in such a young child. We also summarize all cases of angiosarcoma associated with congenital lymphedema reported in the literature.

Asunto(s)

RESUMEN

The aim of the present study was to evaluate the magnetic resonance imaging (MRI) data of patients with Stewart-Treves Syndrome (STS), and to compare them with the corresponding pathological data. A total of 4,289 cases of secondary upper limb lymphedema (LE) from a single center, including 5 female cases of STS who underwent modified-radical post mastectomy, were included in the study. All cases were diagnosed pathologically, and by conventional and contrast-enhanced (ce)MRI scans. A total of 43 tumor nodules (Φ ≥4 mm) were examined. According to the immunohistochemical results, the 5 cases of STS were divided into 2 types; 4 patients exhibited STS type I [angiosarcoma (AS)] and 1 patient exhibited STS type 2 [mixed lymphangiosarcoma (mLAS)]. The MRI signal intensity (SI) of all the nodules was compared with the pathological results. In the T1WI sequences, all nodules showed isointensity compared with normal muscle tissues in the same image. However, in the T2WI sequences, there were the visually recognizable differences in the SI compared with the LE tissues in the two STS types. The SI of mLAS was more markedly decreased compared with that of AS. The SI of mLAS was closer to that of normal muscle tissues compared with the SI of AS. In the ceMRI, the SI of the tumor nodules was markedly increased in the two STS types, compared with non-enhancement imaging. Overall, MRI is a useful tool for the evaluation and diagnosis of STS. A chronic case of LE, in which MRI examination demonstrates nodules within the LE, may suggest a diagnosis of STS.

RESUMEN

Primary angiosarcoma of the breast is extremely rare and is observed in 0.0005% to 0.05% of primary breast tumor cases. The diagnosis of this tumor is difficult due to its undefined characteristics. Radiologic findings are often nonspecific and appear completely normal in one-third of patients with primary angiosarcomas. The prognosis is usually poor, and the treatment choices include mastectomy or wide excision. Radiotherapy and chemotherapy produce varying results. We report a patient with primary angiosarcoma of the breast to further our understanding of the characteristics of this tumor and facilitate the correct diagnosis of breast angiosarcoma.

Asunto(s)

RESUMEN

Abstract: Angiosarcoma is a rare and aggressive tumor with high rates of metastasis and relapse. It shows a particular predilection for the skin and superficial soft tissues. We report three distinct and typical cases of angiosarcoma that were diagnosed in a single dermatology clinic over the course of less than a year: i) Angiosarcoma in lower limb affected by chronic lymphedema, featuring Stewart-Treves syndrome; ii) a case of the most common type of angiosarcoma loated in the scalp and face of elderly man and; iii) a skin Angiosarcoma in previously irradiated breast. All lesions presented characteristic histopathological findings: irregular vascular proliferation that dissects the collagen bundles with atypical endothelial nuclei projection toward the lumen.

Asunto(s)