Malignant alveolar neoplasm in a 10-month-old French bulldog

Background: Malignant tumors are the main cause of death or euthanasia in animals. The oral cavity ranking fourth in number of occurrences. Epidemiological studies with dogs suggest that canine cancer kills 40-50% of individuals aged over 10 years. In view of the interest of academics and professionals in the healthcare of dogs and cats, this paper reports the case of a 10-month-old bitch, which, despite being a young animal, was affected alveolar rhabdomyosarcoma of abrupt evolution. Case: A 10-month-old French Bulldog bitch, weighing 10 kg, was referred to a veterinary hospital in the city of Rio de Janeiro for care. It had a history of mouth bleeding, after chewing a solid mineral material, edema in the region of the right maxilla, and protusion of the gland of the third eyelid. As the clinical examination also revealed a fracture of the maxillary canine, anti-inflammatory and antibiotics were prescribed, to be administered by the owner once a day for 7 days. During the next clinical examination, carried out one week later, an edema was found in the right region of the mouth, which proved difficult to examine. As the patient had already eaten, an appointment was made for the following day for an intervention in the operating room, where the animal could be anesthetized for better observation of the effected region. Blood was collected for hemogram, urea, creatinine, alkaline phosphatase, ALT, and GGT, and an 8 h food fasting and a 4 h water fasting were recommended. On that date, once the dog had been taken to the operating room, was administered the pre anesthesia, in addition to anesthetic induction and manutention. Upon examining the oral cavity, several loose molars were found on the right side, in addition to a tumoral aspect of the gum; thus, it was decided to collect a small sample of the tumoral mass for histopathology. The surgical specimen was placed in a formalin solution and sent to the laboratory for histopathological processing and diagnosis. One week later, the tumor mass was larger and the edema in the right region of the mouth was much larger than on the day of the procedure. Thus, a computerized tomography was requested to further investigate the alterations that had occurred in such a short time. Due to the results of the histopathology and the CT, an immunohistochemical test was suggested which determined the cell profile and morphology and confirmed the diagnosis of alveolar rhabdomyosarcoma according to clinical suspicion. The animal remained in the veterinary hospital for a further 48 h, during which the clinical condition worsened, with the animal suffering heavy bleeding. As the patient was no longer capable of oral intake of food or water, the decision was made with the consent of the owners to induce a painless death to alleviate the suffering of the animal. However, the owners did not authorize a necropsy. Discussion: Veterinary physicians should be conscious of the treatment of serious illnesses that will not result in a benefit for the patient. They should know when to stop the treatment to not cause further pain and suffering to the animals and their owners. Many of the interventions which aim to treat severe malignant neoplasia will not promote an improvement in quality of life or significantly extend the patient's survival, and do not justify the suffering they entail. A painless death remains the best alternative in such cases.

Rabdomiosarcoma alveolar con metástasis cardíaca en un paciente pediátrico / Alveolar rhabdomyosarcoma with cardiac metastasis in a pediatric patient

El rabdomiosarcoma es el tumor maligno de partes blandas más frecuente en la edad pediátrica. Puede afectar cualquier localización anatómica. El subtipo histológico alveolar suele causar lesiones en las extremidades en niños de mayor edad. Los sitios metástasicos más frecuentes son el pulmón, la médula ósea, el hueso y los ganglios linfáticos. Describimos el caso de un paciente con rabdomiosarcoma alveolar (RA) con metástasis cardíaca, una presentación poco frecuente de la patología.

Rhabdomyosarcoma is the most common malignant soft tissue tumor in pediatric age. It can affect any anatomical location. Alveolar histological subtype usually presents lesions on the extremities in older children. The most common metastatic sites are the lung, bone marrow, bone and lymph node. We describe a case of alveolar rhabdomyosarcoma with cardiac metastasis in a pediatric patient, a rare presentation of the pathology.

GEIS-SEHOP clinical practice guidelines for the treatment of rhabdomyosarcoma.

Rhabdomyosarcoma (RMS) is the most frequent soft tissue sarcoma (STS) in children and adolescents. In Spain the annual incidence is 4.4 cases per million children < 14 years. It is an uncommon neoplasm in adults, but 40% of RMS are diagnosed in patients over 20 years of age, representing 1% of all STS in this age group. RMS can appear anywhere in the body, with some sites more frequently affected including head and neck, genitourinary system and limbs. Assessment of a patient with suspicion of RMS includes imaging studies (MRI, CT, PET-CT) and biopsy. All patients with RMS should receive chemotherapy, either at diagnosis in advanced or metastatic stages, or after initial resection in early local stages. Local control includes surgery and/or radiotherapy depending on site, stage, histology and response to chemotherapy. This guide provides recommendations for diagnosis, staging and treatment of this neoplasm.

[Alveolar rhabdomyosarcoma with cardiac metastasis in a pediatric patient]. / Rabdomiosarcoma alveolar con metástasis cardíaca en un paciente pediátrico.

Rhabdomyosarcoma is the most common malignant soft tissue tumor in pediatric age. It can affect any anatomical location. Alveolar histological subtype usually presents lesions on the extremities in older children. The most common metastatic sites are the lung, bone marrow, bone and lymph node. We describe a case of alveolar rhabdomyosarcoma with cardiac metastasis in a pediatric patient, a rare presentation of the pathology.

El rabdomiosarcoma es el tumor maligno de partes blandas más frecuente en la edad pediátrica. Puede afectar cualquier localización anatómica. El subtipo histológico alveolar suele causar lesiones en las extremidades en niños de mayor edad. Los sitios metástasicos más frecuentes son el pulmón, la médula ósea, el hueso y los ganglios linfáticos. Describimos el caso de un paciente con rabdomiosarcoma alveolar (RA) con metástasis cardíaca, una presentación poco frecuente de la patología.

Alveolar rhabdomyosarcoma: origin and prognostic implications of molecular findings / Rabdomiosarcoma alveolar: origen de los hallazgos moleculares e implicaciones pronósticas

Abstract: We present the case of a 2-year-old male patient with a facial tumor partially treated with chemotherapy before his admission to our institution. The tumor involved from the frontal region to the maxillary floor, the orbit, and the maxillary and sphenoid sinuses. The histopathological diagnosis revealed a stage IV alveolar rhabdomyosarcoma with infiltration to bone marrow and cerebrospinal fluid. He was managed with four cycles of adriamycin, actinomycin, cyclophosphamide and vincristine; cisplatin and irinotecan were added to the last cycle. The tumor had a 50% size reduction, but the patient died after a neutropenia and fever episode. The aggressive behavior of alveolar rhabdomyosarcoma has been associated with the expression of oncogenic fusion proteins resulting from chromosomal translocations, particularly t(2;13) (q35;q14) PAX3/FOXO1, and t(1;13) (p36;q14) PAX7/FOXO1 which were present in this patient.

Resumen: Se presenta el caso de un niño de dos años de edad con un tumor facial tratado parcialmente con quimioterapia anterior a su admisión en este hospital. El tumor abarcaba desde la región frontal hasta el piso maxilar, la órbita y los senos esfenoidales y maxilares. El diagnóstico histopatológico reveló un rabdomiosarcoma alveolar estadio IV con infiltración a la médula ósea y fluido cerebroespinal. El paciente fue tratado con cuatro ciclos de adriamicina, actinomicina, ciclofosfamida y vincristina; al último ciclo se añadieron cisplatino e irinotecan. El tumor se redujo en 50% de su tamaño, pero el paciente murió tras un episodio febril y neutropénico. La agresividad del rabdomiosarcoma alveolar se ha asociado con la expresión de proteínas oncogénicas de fusión provenientes de translocaciones cromosomales, particularmente t(2;13) (q35;q14) PAX3/FOXO1 y t(1;13) (p36;q14) PAX7/FOXO1, presentes en este paciente.

Alveolar rhabdomyosarcoma: origin and prognostic implications of molecular findings.

We present the case of a 2-year-old male patient with a facial tumor partially treated with chemotherapy before his admission to our institution. The tumor involved from the frontal region to the maxillary floor, the orbit, and the maxillary and sphenoid sinuses. The histopathological diagnosis revealed a stage IV alveolar rhabdomyosarcoma with infiltration to bone marrow and cerebrospinal fluid. He was managed with four cycles of adriamycin, actinomycin, cyclophosphamide and vincristine; cisplatin and irinotecan were added to the last cycle. The tumor had a 50% size reduction, but the patient died after a neutropenia and fever episode. The aggressive behavior of alveolar rhabdomyosarcoma has been associated with the expression of oncogenic fusion proteins resulting from chromosomal translocations, particularly t(2;13) (q35;q14) PAX3/FOXO1, and t(1;13) (p36;q14) PAX7/FOXO1 which were present in this patient.

Rabdomiosarcoma alveolar de patrón sólido nasal, a propósito de un caso / Pattern alveolar rhabdomyosarcoma nasal solid, on the subject of a case

El rabdomiosarcoma (RMS) es considerado el sarcoma infantil más frecuente, y se origina a partir de células precursoras mesenquimatosas embrionarias del músculo estriado. El compromiso cutáneo se da por metástasis o por invasión local y es poco frecuente. El 41% de los RMS en los niños se localiza en cabeza y cuello, y las formas embrionaria y alveolar son las variantes más frecuentes. Presentamos un caso de RMS nasal de rápido crecimiento variante alveolar de patrón sólido, debido a la presentación en edad temprana para esta variante (AU)

Rhabdomyosarcoma (RMS) is one of the most common pediatric sarcoma, originated of the mesenquimal embryonal smooth muscle precursor cells.This tumor is unusual, when it involves the skin is by metastasis or local invasion. 41% of the pediatric RMS occurs in the head and neck.The alveolar and embryonal forms are the most common variants.We report a case of RMS alveolar form of solid pattern with rapid growth on nasal area (AU)