Clinical, pathological and therapeutic findings on lingual rhabdomyosarcoma in a dog - case report / Achados clínicos, patológicos e terapêuticos do rabdomiossarcoma em cão - relato de caso

The biological behaviour of the tumours vary according to the species in which they occur, its location within the cavity, clinical stage and histopathological nature. Lingual neoplasms are generally uncommon in companion animals. Rhabdomyosarcomas are malignant, solid, aggressive formations with high metastatic potential. The clinical signs are variable and a definitive diagnosis can only be reached through histopathological analysis of biopsy and necropsy specimen. In some cases, immunohistochemical study may be needed to confirm the diagnosis. This paper aims to highlight important points about this uncommon condition in dogs, using a case report of lingual rhabdomyosarcoma, which showed no evidence of metastasis after diagnosis, nor of local recurrence after surgical excision with a wide safety margin. It was concluded that early diagnosis, the correct interpretation of the complementary tests and the appropriate therapeutic approach contributed to improving the quality of life and survival of the patient in question.(AU)

O comportamento biológico dos tumores varia de acordo com a espécie animal, a localização na cavidade, a fase clínica e a natureza histopatológica. As neoplasias orais são geralmente incomuns em animais de companhia. Os rabdomiossarcomas são formações malignas, sólidas e agressivas, com alto potencial metastático. Os sinais clínicos são variáveis e o diagnóstico definitivo só pode ser alcançado por meio da análise histopatológica do material colhido. Em alguns casos, o estudo imuno-histoquímico pode ser necessário para confirmar o diagnóstico. Este trabalho tem como objetivo destacar pontos importantes sobre essa condição incomum em cães, utilizando um relato de caso de rabdomiossarcoma lingual, no qual não se evidenciaram metástases após o diagnóstico, nem recorrência local após a excisão cirúrgica com ampla margem de segurança. Concluiu-se que o diagnóstico precoce, a correta interpretação dos exames complementares e a abordagem terapêutica adequada contribuíram para melhorar a qualidade de vida e a sobrevida do paciente em questão.(AU)

Transformación de teratoma en rabdomiosarcoma / Transformation of teratoma in rabdomiosarcoma

Los teratomas son tumores germinales malignos compuestos por dos o más capas de tejido, que ocasionalmente se transforman en estirpes con crecimiento agresivo. Se presenta el caso de un paciente de 29 años con un tumor germinal gonadal localizado en testículo, cuya evolución fue desfavorable por presentar transformación en un fenotipo correspondiente a un rabdomiosarcoma. La patología aquí descripta deviene del crecimiento diferencial de un componente ya existente en el tumor original o la transformación en un linaje somático que se hace dominante. Los tumores transformados como el del caso descripto son raros y presentan características diferentes de la mayoría de las neoplasias germinales respecto del comportamiento, el pronóstico y la sensibilidad a los tratamientos establecidos.

Teratomas are malign germ cell tumors composed of two or more tissue layers. When there is specific organ differentiation they are called mature teratoma. They rarely grow aggressively. We report the case of a 29 year-old man with a diagnosis of gonadal germ cell tumor whose evolution was unfavorable owing to transformation into a different phenotype corresponding to a rhabdomyosarcoma. This phenomenon occurs through differential growth of a single histological component of the original tumor or transformation of a somatic lineage that becomes dominant. Transformed tumors such as the one herein described differ from most germ cell neoplasms regarding behavior, prognosis, and susceptibility to established treatments.

Rhabdomyosarcoma of Orbit: A Rare Case Report and Review.

Rhabdomyosarcoma (RMS) is a highly fulminant, mesenchymal malignant tumor and is considered to be one among life-threatening disease in the present decades. It is considered to be most common malignant neoplasm of the head and neck region with 10% of cases occurring in orbit. Th ough it is common in sixth and seventh decades, it can also occur in early childhood. In this article, we present a rare case of 9-yearold boy who was diagnosed with histopathologically proven RMS of orbit who had undergone a salvage left orbital exenteration following a chemotherapy. Th e main aim of this article is also to provide an overview of RMS of orbit, clinical features, investigations required, staging and various treatment modalities.

Tumores de testículo em crianças e adolescentes / Testicular tumors in children and adolescents

OBJETIVO: Avaliar comparativamente aspectos clínicos e epidemiológicos dos tumores de testículo na infância e adolescência. MÉTODOS: Análise retrospectiva dos prontuários de pacientes com neoplasias de testículo ou paratesticulares. Foram classificados como crianças os menores de 10 anos e como adolescentes os pacientes entre 10 e 20 anos. Os resultados obtidos foram comparados por meio do teste para duas proporções: teste não paramétrico de Mann-Whitney e teste de log-rank. RESULTADOS: No período de janeiro de 1992 a julho de 2009, foram admitidos 60 pacientes: 34 crianças e 26 adolescentes com neoplasias de testículo ou paratesticulares. As principais manifestações foram tumor e dor na bolsa escrotal. A queixa de dor foi mais comum em adolescentes (p = 0,006). Estes apresentaram tempo médio de história de 4,9 meses, mais prolongado do que crianças, com 2,3 meses (p = 0,01). Os tipos histológicos encontrados foram: tumores de células germinativas em 32/60 (53 por cento), rabdomiossarcomas (RMSs) em 23/60 (38,3 por cento) e outros em 5/60 (8,3 por cento). Os adolescentes apresentaram maior frequência de RMSs, metástases em linfonodos (p = 0,003) e a distância (p = 0,035). As diferenças na sobrevida dos pacientes estudados não foram estatisticamente significantes, havendo apenas indicativo de que a sobrevida, nos casos de RMS, é maior nas crianças (p = 0,072). CONCLUSÕES: Os adolescentes com tumor testicular apresentaram maior tempo de história, tipo histológico agressivo e doença avançada ao diagnóstico quando comparados às crianças, a despeito da pequena amostra.

OBJECTIVE: To perform a comparative assessment of the clinical and epidemiological aspects of testicular tumors in childhood and adolescence. METHODS: Retrospective analysis of medical records of patients with testicular or paratesticular neoplasms. Patients under 10 years were classified as children and patients between 10 and 20 were classified as adolescents. The obtained results were compared through the two-sample test for proportions: non-parametric Mann-Whitney test and log-rank test. RESULTS: 60 patients were admitted in the period from January 1992 to July 2009: 34 children and 26 adolescents with testicular or paratesticular neoplasms. The main manifestations were testicular tumor and scrotal pain. Pain complaints were more common in adolescents (p = 0.006), who presented a mean time from disease onset to diagnosis of 4.9 months, longer than children, who presented a period of 2.3 months from disease onset to diagnosis (p = 0.01). Histological types were as follows: germ cell tumors in 32/60 (53 percent), rhabdomyosarcomas (RMSs) in 23/60 (38.3 percent), and other in 5/60 (8.3 percent). Adolescents presented a higher incidence of RMSs, lymph node metastases (p = 0.003) and distant metastases (p = 0.035). Differences in survival rates among the studied patients were not statistically significant, the only indicative being that survival in RMS cases is longer for children (p = 0.072). CONCLUSIONS: Compared to children, adolescents with testicular tumor presented longer time from disease onset to diagnosis, aggressive histological type and advanced illness at diagnosis, despite the small sample analyzed.

Massive diffuse intraperitoneal multicystic mass in an infant

An 18-month-old boy was referred for painless progressively increasing abdominal distension, poor oral intake, and lethargy since 1 year. There was no history of fever, hematurea, or intestinal obstruction. The child was pale and had a pulse rate of 150 beats/min. The child had mild respiratory distress due to huge abdominal distension. The abdomen was tense and nontender. There was no evidence of intraperitoneal free fluid or any definitive lump. The hemogram showed anemia, other blood biochemistry test results were normal. Ultrasonography of the abdomen suggested the possibility of encysted ascites. A 64-slice contrast-enhanced computed tomography of abdomen showed diffuse intraperitoneal multicystic mass, composed of variable sizes and wall thickness cysts [Figure 1]. The definitive organ of origin could not be identified. Bowel loops were displaced upward by the mass. Diagnostic laparoscopy showed a pale, soft, extremely friable mass composed of cysts of variable sizes arising from the dome of the bladder. The cysts were noncommunicating and had no fluid. The mass was loosely adhered to the bowel, liver, and peritoneum. The rest of the abdominal organs were normal. Complementary cystoscopy was performed to rule out intraluminal extension of tumor, which was normal

Surgical treatment of primary cardiac rhabdomyosarcoma / Tratamento cirúrgico de rabdomiosarcoma primário do coração

The authors report a case of a 16-year-old man who presented progressive dyspnea. At that time the diagnosis of rheumatic fever with mitral valve involvement was performed. The bidimensional echocardiogram showed presence of mobile mass inside the left atrium. The tumor presented lobules, projecting into the left ventricle during the diastole and provoking turbulence. The patient underwent surgical resection with postoperative course needing re-operation for mitral valve replacement. Histopathology has proven that such tumor was a primary cardiac rhabdomyosarcoma and the early clinical diagnosis of rheumatic mitral valve disease was very difficult.

Os autores reportam caso de jovem de 16 anos, o qual apresentou dispnéia progressiva. No momento do atendimento foi feito diagnóstico de febre reumática com comprometimento da valva mitral. Ecocardiograma bidimensional demonstrou a presença de massa móvel dentro do átrio esquerdo. O tumor apresentava lobos, se projetando para o interior do ventrículo esquerdo durante a diástole, provocando turbulência. O paciente foi submetido a ressecção cirúrgica, complicada com reoperação e troca valvar mitral. A histopatologia demonstrou tratar-se de rabdomiosarcoma primário e o diagnóstico clínico diferencial com febre reumática e doença valvar mitral foi muito difícil desde o seu início.

Processos expansivos orbitoesfenoidais: estudo anatomopatológico de 82 casos / Orbito-sphenoidal expansive processes: an anatomopathological study of 82 cases

OBJETIVO: Descrever os processos expansivos orbitoesfenoidais diagnosticados no Laboratório de Anatomia Patológica da Fundação Faculdade Federal de Ciências Médicas de Porto Alegre - Complexo Hospitalar Santa Casa de Porto Alegre durante o período de 15 anos, avaliando sua frequência relativa aos demais processos tumorais. MÉTODOS: Foi realizado levantamento estatístico de todos os tumores de órbita com diagnóstico anatomopatológico durante o período de janeiro de 1968 a dezembro de 1982, e correlacionada a frequência de tumores de órbita com o número total de tumores diagnosticados neste Laboratório por um período de 5 anos. RESULTADOS: Foram diagnosticados 82 casos de processos expansivos que acometiam a órbita no período estudado, sendo 20,7 por cento do total (17 casos) em crianças (até 14 anos) e os 79,3 por cento restantes (65 casos) em adultos. As crianças apresentaram mais frequentemente gliomas de nervo óptico (4 de 6 casos - 66,6 por cento), retinoblastomas (4 casos - 100 por cento) e rabdomiossarcomas (3 de 4 casos - 75 por cento). Outros diagnósticos menos frequentes em crianças foram meningioma do nervo óptico, neurofibroma, pseudotumor inflamatório, dacrioadenite crônica, neuroma e processo inflamatório crônico. Já a população adulta apresentou maior incidência de carcinomas basocelulares (18 casos), carcinomas epidermóides (12 casos), meningiomas (10 casos), melanomas malignos de coróide (3 casos) e tumores de glândula lacrimal (7 casos). Tumores derivados de estruturas ósseas ou vasculares, pseudotumores e cistos epidermóides intraorbitários foram também diagnosticados, entre outros. De um total de 2.639 tumores diagnosticados neste Laboratório no período de 5 anos (1976 a 1980), foram encontrados 22 tumores orbitários, perfazendo 0,8 por cento do total de casos. CONCLUSÕES: O estudo anatomopatológico destes processos é de fundamental importância para o diagnóstico e para o estabelecimento de terapêuticas adequadas. As ...

PURPOSE: To describe the orbito-sphenoidal expansive processes diagnosed at the Anatomo-Pathological Laboratory of the Fundação Faculdade Federal de Ciências Médicas de Porto Alegre - Complexo Hospitalar Santa Casa de Porto Alegre during a period of 15 years, evaluating their relative frequencies among other tumoral processes. METHODS: We performed a statistical analysis of all orbital tumors with anatomicopathological diagnosis from January 1968 to December 1982, comparing the frequency of orbital tumors with the total number of tumors diagnosed at this Laboratory in a period of 5 years. RESULTS: Eighty-two cases were diagnosed of expansive processes involving the orbit in 15 years - 20.7 percent of the total (17 cases) affecting children (up to 14 years-old) and the remaining 79.3 percent (65 cases) affecting adults. Children presented more frequently optic nerve gliomas (4 of 6 cases 66.6 percent), retinoblastomas (4 cases - 100 percent) and rhabdomiosarcomas (3 of 4 cases - 75 percent). Less frequent diseases in children were optic nerve meningioma, neurofibroma, inflammatory pseudotumor, chronic dacryoadenitis, neuroma and chronic inflammatory process. The adult population presented more cases of basal cell carcinomas (18 cases), squamous cell carciomas (12 cases), meningiomas (10 cases), choroidal malignant melanoma (3 cases) and lacrimal gland tumors (7 cases). Tumors originated from bone or vascular structures, pseudo-tumors, and intraorbital epidermic cysts were also diagnosed, among others. Of a sum of 2,639 tumors diagnosed at this Laboratory in the time period of 5 years (1976 to 1980), there were 22 cases of orbital tumors, reaching a total of 0.8 percent of all cases. CONCLUSIONS: The anatomicopathological study of these processes is somehow important to diagnose and to establish an adequate therapy. The incidence of the expansive processes involving the orbit allows an epidemiological characterization of the different medical ...

Massive bone marrow involvement by clear cell variant of rhabdomyosarcoma.

A 16-year-old boy with generalized myalgia and petechial hemorrhage was found to have a diffuse infiltrative disease in his bone marrow (BM). The BM aspirate contained a dense population of vacuolated blast-like cells. The BM biopsy displayed compact sheets of small round cells with clear cytoplasm, reminiscent of Ewing sarcoma. Immunostains were not diagnostically conclusive while transmission electron microscopy on the BM cells demonstrated a clear skeletal muscle differentiation. The morphologic findings led to a tentative designation of metastatic embryonal rhabdomyosarcoma (RMS). It was not until cytogenetic analysis revealed the specific translocation t(2;13)(q35;q14) did the alveolar RMS finally get confirmed. Despite an exhaustive search by imaging studies, a primary tumor was not detected. This case illustrates that the massive BM involvement by atypical alveolar RMS of unknown origin may pose serious diagnostic challenges. Multidisciplinary studies are required to reach a definitive diagnosis.

Frequency of bone invasion and metastasis in round cell tumours of paediatric age groups

In this study a total of 14 patients of paediatric age group were included with round cell tumours of Retinoblastoma, Neuroblastoma, Ewings's sarcoma and Rhabdomyosarcoma. The frequency of metastatic involvement of the bone marrow was determined by aspiration and biopsy from posterior iliac crest. Routine investigations like haemoglobin, total leukocyte count and, platelets count were also carried out in all cases. Results show that the neuroblastoma was most common to metastasize to bone marrow. It was also noted that, aspirate and biopsy were found complementary in diagnosis of metastasis. However further work is needed to find out the frequency of metastatic involvement of bone marrow in a larger series of cases

Rhabdomyosarcoma of the nasal vestibule in a child

A case of rhabdomyosarcoma in a 2 year old girl without a pre-existing predisposing factor visited the ENT Department of Sultan Qaboos University Hospital [SQUH]. Her clinical condition did not point to the diagnosis, which came as a histological surprise. The histopathology report was alveolar rhabdomyosarcoma

Immunophenotypic characterization of high grade pleomorphic sarcomas: a demographic and immunohistochemical study in a major referral center of Pakistan

Immunophenotypic characterization of high grade [pleomorphic] sarcomas and determination of their frequency, mean/median age, sex preferences and common sites in Pakistani patients. This study included 134 consecutive cases of high grade [pleomorphic] sarcomas diagnosed in adults above the age of 15 years in the section of histopathology at the Aga Khan University Hospital, Karachi during a period of two years. These high grade [pleomorphic] sarcomas were immunophenotyped using a panel including antibodies against Vimentin, Desmin, Smooth muscle Actin, S 100, CD34, CD68 and Cytokeratin etc by indirect immunoperoxidase. Of the 134 cases which were characterized, 38.1% were pleomorphic leiomyosarcoma, followed by pleomorphic rhabdomyosarcoma 14.9%, Malignant Peripheral Nerve Sheath Tumour 9%, pleomorphic liposarcoma 3.7% and pleomorphic storiform Malignant Fibrous Histiocytoma 0.7%. Thirty three% of cases could not be characterized further. Mean/ median age for Leiomyosarcoma was 50/50, for Rhabdomyosarcomas 33/22, for MPNST 42/41, for Liposarcoma 52/50 and for Malignant Fibrous Histiocytoma 46/46 respectively. The commonest site for leiomyosarcoma was lower limb [43%], for rhabdomyosarcoma head and neck [42%], for MPNST thorax [36.4%] and for liposarcoma abdomen [50%]. It was concluded that the most common pleomorphic sarcoma occurring in our adult population was Leiomyosarcoma, and that immunohistochemical stains are essential in most cases for further characterization of pleomorphic high grade sarcoma

[Small round cell tumours with unknown origin in pathology specimens of iman reza hospital from 1362-1382]

The term" small round cell tumour"describes a group of highly aggressive tumours composed of relatively small and monotonous undifferentiated cells with high nuclear to cytoplasmic ratio. This group includes: Ewing sarcoma [EWS], Peripheral neuroepithelioma [PN], Primitive neuroectodermal tumour [PNET], Neuroblastoma, Rhabdomyosarcoma [RMS], Desmoplastic small round cell tumour [DSRCT], Lymphoma, Leukemia, Small cell osteosarcoma Small cell carcinoma, Olfactory neuroblastoma, Merkel cell carcinoma, Small cell melanoma, and Mesenchymal chondrosarcoma. Their clinical presentation often overlap, thus making a definitive diagnosis problematic in some cases. Yet, a clear undrestanding of their clinicopathologic features usually allows for a confident diagnosis, especially if immunohistochemistry is used. This is an immunohistochemistry study of small round cell tumours with unknown origin that were diagnosed in pathology service of Imam Reza Hospital from 1362-1382. In this period, we found 24 cases which were reported SRCT without definitive diagnosis the immunohistochemistry kits are from DAKO Company and applied as directed by manufacturers. The SRCT is most frequent in the first decade of life [45%], 13 cases were male and 11 cases were female. IHC influence on diagnosis in 11 cases [46%] confirm the first ordered diagnosis and diagnosis made in another 9 [33%] of SRCTs,and in 2cases[8%]the first diagnosis was changed. Overally in review, 35 cases from 48 SRCTs with unknown origin [%75] were diagnosed by routine stainings by light microscopy without immunohistochemistry

Prepubertal testicular tumours in Kashmir--a histopathological report of 15 cases.

A total of 15 cases of prepubertal testicular tumours were reported by the department of pathology, Government Medical College, Srinagar, Kashmir over a period of 15 years, from January 1984 to December 1998. The cases included yolk sac tumour (10 cases; 67%), teratoma [(mature) 2 cases (13%)], rhabdomyosarcoma [(paratesticular) 2 cases (13%)] and NHL-Burkitt's lymphoma [one case (7%)]. The youngest patient was 10 months old and the oldest was of 14 years age. Ten cases (67%) occurred at or below the age of 4 years. The youngest patient had yolk sac tumour and oldest had rhabdomyosarcoma. In 2 cases both the testes were involved with one of these two cases having bilateral undescended testes.

Molecular biology and genetics affecting pediatric solid tumors

Since the discovery of oncogenes more than 20 years ago, it has been proven that cancer is a genetically determined disease. Multiple genetic alteration occurs during the course of an illness for neoplasia to develop. Transformation of positive cell growth regulators (oncogenes) and inactivations of negative cell growth regulators (tumor suppressor genes) merge to express a malignant phenotype. These genetic alterations occur as chromosomal translocations, deletions, inversion, amplification or point mutation. The objective of this review is to introduce basic concepts of molecular biology and describe the molecular genetics and biologic clinical findings of the most important solid malignant tumors in children, namely Neuroblastoma, Wilms and Rhabdomyosarcoma. It is the oncology surgeons responsibility to learn basic molecular genetics and tumor biology to provide rational and appropriate care in the setting of multidisciplinary management. Identifications of new oncogenes will continue to be important milestones in diagnosis, early detection of tumor recurrence, and as potential targets for gene therapy. Fusion proteins generated by mutated translocations are true tumor specific antigens and potential targets for therapy. The predicament is that they are proteins needing therapeutic manipulation within the tumor cell nuclei. Technological advances in molecular and genetics will develop tools necessary to manipulate the cell nuclear DNA and target cancer cell

Rabdomiosarcoma en la región cervicofacial: informe de dos casos / Cervicofacial rhabdomyosarcoma: report of two cases

Los rabdomiosarcomas (RMS) son los sarcomas de tejidos blandos más comunes en niños y 15 por ciento de los tumores sólidos tratados a nivel hospitalario en servicios pediátricos; se presentan con una amplia variedad morfológica, en diferentes órganos y con una gama de patrones histológicos, lo que puede dificultar su diagnóstico. Se reportan dos casos de rabdomiosarcoma, uno embrionario y otro alevolar, que se presentaron en la región preauricular y maseterina, respectivamente, en pacientes masculinos, diagnosticados y tratados en el Hospital Central Dr. Ignacio Morones Prieto SSA San Luis Potosí, SLP