Chemodectoma in a bitch

Background: Chemodectomas, better known as tumors of the base of the heart, arise from aortic bodies, respiratory chemoreceptors located near or inside the aortic arch or originate from receptors located in the carotid arteries. Relatively rare, they affect dogs and, to a lesser extent, cats. They gain great importance when they influence the function of the cardiovascular system, with animals showing clinical signs related to congestive heart failure. Clinical diagnosis is based on symptomatology and complementary tests such as radiography, electrocardiography and echocardiography, while the definitive diagnosis is obtained by cytological and histopathological exams. This study aims to reports a case of malignant chemodectoma in a bitch, whose main symptomatology was neurological and not cardiovascular. Case: A 1-year-old Rottweiler bitch was attended with neurological alterations compatible with vestibular syndrome, hyporexia, dysphagia, apathy, melena, emesis, and purulent nasal discharge on the right nostril. On physical examination, the animal showed depressed level of consciousness, poor body condition, bilateral quemosis, paralysis of the right eye, inspiratory dyspnea and muffling of cardiac auscultation, besides a subcutaneous nodule between the scapulae. On neurological evaluation, horizontal nystagmus, head tilt to the right side, ventromedial strabismus and facial nerve paralysis on the right side were observed so that the localization of the lesion was set in peripheral vestibular system. During anesthesia for esophageal tube placement, a mass from the hard palate to the oropharynx was noted, making endotracheal intubation impossible to perform. Biopsy of this nodule was performed, and tracheostomy was indicated, but the owner opted for euthanasia before the procedure. Necropsy revealed white soft masses in the bilateral retromandibular region, on the subcutaneous tissue near the scapulae, in the right ear and since nasopharynx to the soft palate, in addition to sparse white nodules in the heart, lung, carotid artery, kidneys, right ovary, mesentery near to the spleen, and axillary lymph node. Histologically, the nodules were characterized by neoplastic cells population organized in short bundles or cords, arranged around small blood vessels surrounded by delicate connective tissue. Neoplastic cells infiltrated muscles and blood and lymphatic vessels were filled by multiple neoplastic emboli. The histological pattern of the cells allowed the diagnosis of chemodectoma. Discussion: The bitch from this case had 1-year-old when diagnosed with chemodectoma, differently from most cases from literature, that are between 7 to 15 years old. Furthermore, primarily cardiac tumors are considered rare, being chemodectoma the most common, often reported in Boxer and Boston Terrier dogs, but unusual in Rottweilers. Despites some articles mentioning seizure and Horner's Syndrome secondary to a carotid body chemodectoma, neurological signs are not commonly observed in these cases. The presence of the tumor in the middle ear region of the right side supports the occurrence of peripheral vestibular syndrome and facial nerve paralysis on the same side. Because it is a neoplasm that is usually detected late during the course of the disorder, most patients either cannot obtain diagnosis in vivo, as in this reported animal, which was in such a critical condition that underwent euthanasia, or there are no more possible therapeutic choices. In the patient described, there were numerous metastatic masses and nodules spread throughout the body. Although the typical clinical signs in animals with chemodectomas are often related to heart disease, neurological signs may also be present. This report emphasizes the importance of chemodectoma being included as a differential diagnosis in young dogs and even in breeds such as Rottweiler.

Quimiodectoma em cão / Chemodectoma in a dog

Background: The chemodectoma is a rare neoplasm that originates from chemoreceptors located mainly in the aortic body,and carotid body and sinus, and responsible for detecting variations in blood pH, oxygen pressure and carbon dioxide. Dogsof brachiocephalic breeds and aged between 7 and 15 years have greater propensity. A neoplasm involves infiltrative growthin the vessels at the heart base, which leads to Congestive Heart Failure (CHF). The definitive diagnosis is performed byhistopathological and/or immunohistochemical examination. The aim was to report a case of chemiodectoma in a dog,showing the diseases clinical characteristics.Case: A 13-year-old male undefined breed dog was examinated in the medical clinic of small animal of Veterinary Hospital,Federal Rural University of Pernambuco (UFRPE) with respiratory effort, hyporexia, and edema in face, cervical, ventralthorax and thoracic limbs, with thirty days evoluted. About physical examination, we observed cachexia, orthopneic position, cyanosis and edema with a positive Godet sign, as well as 8% dehydration degree. Thoracic auscultation presentedmixed dyspnea and muffled heart sounds. Chest radiography detected an radiopacity increase in pulmonary section andmetastatic neoplastic process associated with pleural and pericardial effusion. Fluid therapy with lactated ringer and posteriorthoracentesis in the right hemithorax region was performed for greater respiratory comfort for the patient. Hematologicalcount and biochemical profiles stated normochromic normocytic anemia, relative and absolute lymphopenia, thrombocytopenia, as well as increased alanine aminotransferase (ALT) and aspartate aminotransferase (AST). The patient diedafter 12 hours when was submitted to necroscopic examination and histopathological evaluation. An infiltrative tumor ofcardiac base was observed invading the light of the right atrium, obstructing the venous return, as well as a large thrombus...(AU)

Quimiodectoma em cão / Chemodectoma in a dog

Background: The chemodectoma is a rare neoplasm that originates from chemoreceptors located mainly in the aortic body,and carotid body and sinus, and responsible for detecting variations in blood pH, oxygen pressure and carbon dioxide. Dogsof brachiocephalic breeds and aged between 7 and 15 years have greater propensity. A neoplasm involves infiltrative growthin the vessels at the heart base, which leads to Congestive Heart Failure (CHF). The definitive diagnosis is performed byhistopathological and/or immunohistochemical examination. The aim was to report a case of chemiodectoma in a dog,showing the disease’s clinical characteristics.Case: A 13-year-old male undefined breed dog was examinated in the medical clinic of small animal of Veterinary Hospital,Federal Rural University of Pernambuco (UFRPE) with respiratory effort, hyporexia, and edema in face, cervical, ventralthorax and thoracic limbs, with thirty days evoluted. About physical examination, we observed cachexia, orthopneic position, cyanosis and edema with a positive Godet sign, as well as 8% dehydration degree. Thoracic auscultation presentedmixed dyspnea and muffled heart sounds. Chest radiography detected an radiopacity increase in pulmonary section andmetastatic neoplastic process associated with pleural and pericardial effusion. Fluid therapy with lactated ringer and posteriorthoracentesis in the right hemithorax region was performed for greater respiratory comfort for the patient. Hematologicalcount and biochemical profiles stated normochromic normocytic anemia, relative and absolute lymphopenia, thrombocytopenia, as well as increased alanine aminotransferase (ALT) and aspartate aminotransferase (AST). The patient diedafter 12 hours when was submitted to necroscopic examination and histopathological evaluation. An infiltrative tumor ofcardiac base was observed invading the light of the right atrium, obstructing the venous return, as well as a large thrombus...

El paraganglioma como tumor mesentérico infrecuente / Mesenteric paraganglioma: a rare tumor

RESUMEN Los paragangliomas son tumores originados en las células neuroendocrinas que forman el sistema nervioso autónomo. Se consideran benignos aunque pueden desarrollar malignidad, por lo que su tra tamiento es quirúrgico. La presentación de paraganglioma de ubicación mesentérica es muy inusual.

ABSTRACT Paragangliomas are rare neuroendocrine tumors that arise in the autonomic nervous system. Although these tumors are considered benign, they must be removed by surgery due to their potential malig nant transformation. Mesenteric paragangliomas are extremely rare.

Hemangiossarcoma associado a quimiodectoma em cadela Pit Bull idosa / Hemangiosarcoma associated with chemodectoma in an old Pit Bull bitch

Background: The prevalence of neoplasms in domestic animals has been augmenting over the years. Hemangiosarcoma, which most frequently affects large breed dogs, is a neoplasm with high metastatic power and corresponds to 5% of malignant neoplasms. Chemodectoma, a neoplasm that can have malignant or benign behavior, is uncommon, corresponding to less than 8% of all cardiac tumors, and originates from cardiac chemoreceptor cells and involves mainly the aortic and carotid bodies. The aim of this study is to describe the case of an elderly Pit Bull dog with hepatic hemangiosarcoma and chemodectoma associated with four other different tumors: hemangioma, papilloma, mastocitoma and melanoma. Case: A 14-year-old female Pit Bull, castrated, was presented to Veterinary Clinic of the Federal University of Santa Catarina (UFSC), Curitibanos Campus, Brazil. The owner referred apathy, hyporexia, and increased abdominal volume for two weeks. Physical examination showed emaciation and abdominal effusion. Abdominocentesis was performed, three liters of serosanguineous fluid was drained and a sample was sent for analysis. Complementary tests were requested and showed mild hypoalbuminemia, normocytic normochromic anemia, and lymphopenia. The ultrasound examination revealed thickening of vessels and hepatomegaly, in addition to the presence of an oval structure containing hyperechogenic cavitations. Diagnostic exploratory laparotomy was indicated, but the owner refused. Drug therapy was prescribed in a palliative manner and, during the clinical follow-up period, two abdominocentesis procedures and one thoracentesis procedure were performed, both resulting in simple transudate. Finally, the patient developed severe apathy, edema in all limbs, and died at home. Necroscopic examination was done and revealed hemoperitoneum and hemothorax...(AU)

Hemangiossarcoma associado a quimiodectoma em cadela Pit Bull idosa / Hemangiosarcoma associated with chemodectoma in an old Pit Bull bitch

Background: The prevalence of neoplasms in domestic animals has been augmenting over the years. Hemangiosarcoma, which most frequently affects large breed dogs, is a neoplasm with high metastatic power and corresponds to 5% of malignant neoplasms. Chemodectoma, a neoplasm that can have malignant or benign behavior, is uncommon, corresponding to less than 8% of all cardiac tumors, and originates from cardiac chemoreceptor cells and involves mainly the aortic and carotid bodies. The aim of this study is to describe the case of an elderly Pit Bull dog with hepatic hemangiosarcoma and chemodectoma associated with four other different tumors: hemangioma, papilloma, mastocitoma and melanoma. Case: A 14-year-old female Pit Bull, castrated, was presented to Veterinary Clinic of the Federal University of Santa Catarina (UFSC), Curitibanos Campus, Brazil. The owner referred apathy, hyporexia, and increased abdominal volume for two weeks. Physical examination showed emaciation and abdominal effusion. Abdominocentesis was performed, three liters of serosanguineous fluid was drained and a sample was sent for analysis. Complementary tests were requested and showed mild hypoalbuminemia, normocytic normochromic anemia, and lymphopenia. The ultrasound examination revealed thickening of vessels and hepatomegaly, in addition to the presence of an oval structure containing hyperechogenic cavitations. Diagnostic exploratory laparotomy was indicated, but the owner refused. Drug therapy was prescribed in a palliative manner and, during the clinical follow-up period, two abdominocentesis procedures and one thoracentesis procedure were performed, both resulting in simple transudate. Finally, the patient developed severe apathy, edema in all limbs, and died at home. Necroscopic examination was done and revealed hemoperitoneum and hemothorax...

[Carney triad: Report of one case]. / Tríada de Carney. Una rara asociación de tumores infrecuentes: Caso clínico.

Carney described a disorder characterized by the presence of several uncommon tumors which were pulmonary chondromas, gastric sarcomas and extra-adrenal paragangliomas. We report a 14 year-old girl in whom multiple gastric tumors were discovered during a study of an iron deficiency anemia and was subjected to a partial gastrectomy. At 25 years of age, she developed several pulmonary chondromas and at 33 years, a mediastinal tumor with features of an extra-adrenal paraganglioma was found. At 35 years of age, a total gastrectomy was performed to remove a gastrointestinal stromal tumor with excision of peritoneal and lymph node metastasis. One year later, the patient died due to liver failure secondary to liver metastases.

Tríada de Carney. Una rara asociación de tumores infrecuentes: caso clínico / Carney triad: report of one case

Carney described a disorder characterized by the presence of several uncommon tumors which were pulmonary chondromas, gastric sarcomas and extra-adrenal paragangliomas. We report a 14 year-old girl in whom multiple gastric tumors were discovered during a study of an iron deficiency anemia and was subjected to a partial gastrectomy. At 25 years of age, she developed several pulmonary chondromas and at 33 years, a mediastinal tumor with features of an extra-adrenal paraganglioma was found. At 35 years of age, a total gastrectomy was performed to remove a gastrointestinal stromal tumor with excision of peritoneal and lymph node metastasis. One year later, the patient died due to liver failure secondary to liver metastases.

Paraganglioma de corpo aórtico em cão / Aortic body paraganglioma in dog

Background: Aortic body paragangliomas are uncommon neoplasms that develop mainly in aortic and carotid bodies. It has been supposed that genetic factors and chronic hypoxia may stimulate tumor development. The brachycephalic dog breeds, as Boxer, are most predisposed to present this neoplasm. The clinical symptomatology is related to tumor size and localization. Usually aortic body paraganglioma has benign biological behavior, when it is malignant, rarely promotes metastases. The aim of this study was to report a case of the aortic body paraganglioma as death cause in a dog. Case: A canine, 10-year-old, male, cross breed, presented clinical signs as anorexia, emesis, cough, dyspnea and exercise intolerance. After death the animal was examined at the Department of Veterinary Pathology at the Federal Rural University of Amazonia. On necropsy, no pericardial effusion was identified, however pleural and abdominal effusion was observed, volume like 1000 and 700 mL, respectively. The heart had a neoplasm near the left atrium, it measured 6.5 x 8.2 cm, had irregular surface, firm consistency, grayish color, and at the cut showed infiltration in the myocardium, as well as obstruction of the left atrial lumen and left ventricle concentric hypertrophy. No distant metastases were found. Microscopically, the tumor consisted of polyhedral morphology cells, eosinophilic cytoplasm, spherical and hyperchromatic nucleus. Cells were grouped into lobes separated by fibrovascular stroma, large cells (less uniform cells), low mitotic rate and myocardial infiltration. On immunohistochemical analysis anti-cytokeratin, anti-vimentin and anti-S-100 antibodies were used. Tumour cells stained was absent for anti-cytokeratin and anti-vimentin, but was anti-S-100 positive. A case of malignant aortic body paraganglioma grade II was diagnosed.[…]

Paraganglioma de corpo aórtico em cão / Aortic body paraganglioma in dog

Background: Aortic body paragangliomas are uncommon neoplasms that develop mainly in aortic and carotid bodies. It has been supposed that genetic factors and chronic hypoxia may stimulate tumor development. The brachycephalic dog breeds, as Boxer, are most predisposed to present this neoplasm. The clinical symptomatology is related to tumor size and localization. Usually aortic body paraganglioma has benign biological behavior, when it is malignant, rarely promotes metastases. The aim of this study was to report a case of the aortic body paraganglioma as death cause in a dog. Case: A canine, 10-year-old, male, cross breed, presented clinical signs as anorexia, emesis, cough, dyspnea and exercise intolerance. After death the animal was examined at the Department of Veterinary Pathology at the Federal Rural University of Amazonia. On necropsy, no pericardial effusion was identified, however pleural and abdominal effusion was observed, volume like 1000 and 700 mL, respectively. The heart had a neoplasm near the left atrium, it measured 6.5 x 8.2 cm, had irregular surface, firm consistency, grayish color, and at the cut showed infiltration in the myocardium, as well as obstruction of the left atrial lumen and left ventricle concentric hypertrophy. No distant metastases were found. Microscopically, the tumor consisted of polyhedral morphology cells, eosinophilic cytoplasm, spherical and hyperchromatic nucleus. Cells were grouped into lobes separated by fibrovascular stroma, large cells (less uniform cells), low mitotic rate and myocardial infiltration. On immunohistochemical analysis anti-cytokeratin, anti-vimentin and anti-S-100 antibodies were used. Tumour cells stained was absent for anti-cytokeratin and anti-vimentin, but was anti-S-100 positive. A case of malignant aortic body paraganglioma grade II was diagnosed.[…](AU)

Quimiodectoma em equino: relato de caso / Chemodectoma in equine: case report / Quimiodectoma en equina: presentación de un caso

O quimiodectoma corresponde à neoplasia dos quimiorreceptores periféricos que detectam alterações de pH, temperatura e pressões parciais de O2 e CO2 na corrente sanguínea. A partir desta detecção, esses transmitem impulsos nervosos para os centros cerebrais respiratórios e cardíacos para promoção da homeostase, através do sistema nervoso autônomo. Em levantamento bibliográfico há relato de apenas dois casos em equinos, um no corpo aórtico de uma fêmea de 5 anos e em um macho castrado de 13 anos de idade. O objetivo do presente trabalho é relatar um caso de quimiodectoma em equino diagnosticado em necropsia no Hospital Veterinário de Uberaba.(AU)

The chemodectoma corresponds to the peripheral chemoreceptors neoplasia which detect changes in pH, temperature and partial pressures of 02 and C02 in the bloodstream. From this detection, these transmit nerve impulses to the brain respiratory and cardiac centers to promote homeostasis, through the autonomic nervous system. In literature there are reports of only two cases in horses, one in aortic body of a female of 5 years and in a castrated male 13 years old. The aim of this study is to report a case of chemodectomaequinus diagnosed at autopsy at the Veterinary Hospital of Uberaba.(AU)

El quimiodectoma corresponde a la neoplasia de los quimiorreceptores periféricos que detectan alteraciones de pH, temperatura y presiones parciales de O2 y CO2 en la corrí ente sanguínea. A partir de esta detección, estos transmiten impulsos nerviosos para los centros cerebrales, respiratorios y cardíacos para promoción de la homeostase, através del sistema nervioso autónomo. En el levantamiento bibliográfico, hay relatos de apenas dos casos en equinos, uno en el cuerpo aórtico de una hembra de 5 anos yotro en un macho castrado de 13 anos de edad. El objetivo del presente trabajo es relatar un caso de quimiodectoma en equino diagnosticado en necropsia en el Hospital Veterinário de Uberaba.(AU)

Quimiodectoma em equino: relato de caso / Chemodectoma in equine: case report / Quimiodectoma en equina: presentación de un caso

O quimiodectoma corresponde à neoplasia dos quimiorreceptores periféricos que detectam alterações de pH, temperatura e pressões parciais de O2 e CO2 na corrente sanguínea. A partir desta detecção, esses transmitem impulsos nervosos para os centros cerebrais respiratórios e cardíacos para promoção da homeostase, através do sistema nervoso autônomo. Em levantamento bibliográfico há relato de apenas dois casos em equinos, um no corpo aórtico de uma fêmea de 5 anos e em um macho castrado de 13 anos de idade. O objetivo do presente trabalho é relatar um caso de quimiodectoma em equino diagnosticado em necropsia no Hospital Veterinário de Uberaba.

The chemodectoma corresponds to the peripheral chemoreceptors neoplasia which detect changes in pH, temperature and partial pressures of 02 and C02 in the bloodstream. From this detection, these transmit nerve impulses to the brain respiratory and cardiac centers to promote homeostasis, through the autonomic nervous system. In literature there are reports of only two cases in horses, one in aortic body of a female of 5 years and in a castrated male 13 years old. The aim of this study is to report a case of chemodectomaequinus diagnosed at autopsy at the Veterinary Hospital of Uberaba.

El quimiodectoma corresponde a la neoplasia de los quimiorreceptores periféricos que detectan alteraciones de pH, temperatura y presiones parciales de O2 y CO2 en la corrí ente sanguínea. A partir de esta detección, estos transmiten impulsos nerviosos para los centros cerebrales, respiratorios y cardíacos para promoción de la homeostase, através del sistema nervioso autónomo. En el levantamiento bibliográfico, hay relatos de apenas dos casos en equinos, uno en el cuerpo aórtico de una hembra de 5 anos yotro en un macho castrado de 13 anos de edad. El objetivo del presente trabajo es relatar un caso de quimiodectoma en equino diagnosticado en necropsia en el Hospital Veterinário de Uberaba.

Management of Carotid Bifurcation Tumors: 30-Year Experience.

BACKGROUND: The carotid bifurcation can host a variety of tumors requiring complex surgical management. Treatment requires resection and, in some cases, vascular reconstruction that may compromise the cerebral circulation. The most frequent lesion at this location is the carotid body tumor (CBT). CBT are classified according to Shamblin in 3 types depending on the degree of carotid vessels encasement. Our main objective was to report our clinical experience managing carotid bifurcation tumors throughout the last 30 years. METHODS: Between 1984 and 2014, we treated 30 patients with 32 carotid bifurcation tumors. There were 21 women and 9 men (2.3:1), with a mean age of 45.5 years (18-75). The most frequent presentation was an asymptomatic neck swelling or palpable mass localized at the carotid triangle (86.7%). RESULTS: Thirty of 32 tumors were resected. Since 1994, computed tomography scan has been the most frequently used diagnostic imaging tool (80%), followed by magnetic resonance imaging. Angiography was used mainly during the first 10 years of the study period. Mean size of the tumor was 44.6 mm (20-73 mm). Nineteen (63%) were classified as Shamblin II and 6 (20%) as Shamblin's III. All specimens were analyzed by a pathologist; 28 tumors (93%) were confirmed as paragangliomas, 2 (7%) were diagnosed as schwannomas. Two patients underwent preoperative embolization of the CBT; 5 patients (17%) required simultaneous carotid revascularization, all of them Shamblin III. Mean hospitalization time was 4.5 days (1-35 days). Transient extracranial nerve deficit was observed in 7 patients (23.3%). Three patients (Shamblin III) required red blood cells transfusion. One patient (Shamblin III) underwent a planned en bloc excision of the vagus nerve. There was no perioperative mortality or procedure-related stroke. No malignancy or tumor recurrence were observed during follow-up. CONCLUSIONS: CBTs can be diagnosed on clinical grounds requiring vascular imaging confirmation. These infrequent lesions are generally benign. Early surgical removal by surgeons with vascular expertise avoids permanent neurologic and or vascular complications.

Hipertensión secundaria a paraganglioma: presentación de un caso y revisión de la literatura / Hipertensão secundária paraganglioma: relato de caso e revisão da literatura / Hypertension secondary to paraganglioma: case report and review of the literature

Se presenta el caso de un hombre de 25 años, con historia de accidente cerebrovascular transitorio durante la adolescencia, e hipertensión arterial no controlada de larga data, que consultó por palpitaciones, diaforesis y cefalea holocraneana. Se le diagnosticó y confirmó por histopatología un paraganglioma paraórtico hipersecretor de catecolaminas. Los síntomas se resolvieron completamente después de la extracción del tumor. El paraganglioma es un tumor extraadrenal infrecuente derivado de las células cromafines; se lo considera como un diagnóstico relevante en la evaluación de la hipertensión arterial secundaria. Se presenta una revisión de la literatura de casos de paragangliomas hipersecretores de catecolaminas cuyo síntoma asociado fue la hipertensión arterial...

We report the case of a 25 year-old man with a history of transient stroke during adolescence, and longstanding uncontrolled hypertension who presented with palpitations, diaphoresis and headache. He was diagnosed with a paraaortic paraganglioma associated with catecholamine hypersecretion, confirmed by histopathology. There was complete resolution of the symptoms after removal of the tumor. Paraganglioma, a rare extra-adrenal tumor, derived from chromaffin cells, should be considered in the diagnosis of secondary hypertension. We performed a literature review of paraganglioma cases with catecholamine hypersecretion, and associated hypertension...

Nós relatamos o caso de um homem de 25 anos com história de acidente vascular cerebral transitória durante a adolescência, e de longa data hipertensão não controlada que se apresentou com palpitações, sudorese e dor de cabeça. Ele foi diagnosticado com um paraganglioma parágrafo aórtica associada a catecolamina hipersecreção, confirmado pelo exame histopatológico. Houve resolução completa dos sintomas, após a remoção do tumor. Paraganglioma, um tumor extra-adrenal rara, derivada a partir de células de cromafina, devem ser considerados no diagnóstico de hipertensão secundária. Foi realizada uma revisão da literatura de casos de paraganglioma com hipersecreção de catecolaminas, e hipertensão associada...

Type 2 segmental glomangioma--Case report.

Glomus tumors originate from modified perivascular muscle cells. The most common form is the solitary one. The multiple form may be associated with dominant genetic inheritance. We report a case of a patient with hemangiomatous lesions on the calcaneus and wrist since birth. In 6 years, there was progression of lesions throughout the body. Multiple glomangiomas are asymptomatic and more common in childhood. They can be confused with other vascular malformations. Histopathological diagnosis is essential. The case shows a type 2 segmental manifestation that can be explained by genetic mutation leading to the loss of heterozygosity. As the child grows, the lesions may disseminate due to mutation in distant parts of the skin. Literature shows few reports. The treatment is conservative.

Type 2 segmental glomangioma - Case report

Glomus tumors originate from modified perivascular muscle cells. The most common form is the solitary one. The multiple form may be associated with dominant genetic inheritance. We report a case of a patient with hemangiomatous lesions on the calcaneus and wrist since birth. In 6 years, there was progression of lesions throughout the body. Multiple glomangiomas are asymptomatic and more common in childhood. They can be confused with other vascular malformations. Histopathological diagnosis is essential. The case shows a type 2 segmental manifestation that can be explained by genetic mutation leading to the loss of heterozygosity. As the child grows, the lesions may disseminate due to mutation in distant parts of the skin. Literature shows few reports. The treatment is conservative.