Radioterapia neoadyuvante e intraoperatoria en sarcoma de muslo y región poplítea para salvamento de extremidad. Reporte de caso y revisión de la literatura / Preoperative and intraoperative radiotherapy for limb sparing surgical resection in a popliteal fossa sarcoma. Case report and review of the literature

Resumen Los sarcomas de tejidos blandos de las extremidades necesitan para su manejo la realización de resecciones oncológicas amplias con el fin de lograr márgenes negativos. En ocasiones los casos con compromiso de estructuras neurovasculares y/u óseas requieren la amputación de la extremidad como única alternativa quirúrgica. Presentamos el caso de un paciente con diagnóstico de liposarcoma que comprometía la fosa poplítea y el paquete neurovascular. Recibió radioterapia neoadyuvante, resección compartimental y radioterapia intraoperatoria, que posibilitaron la conservación de la extremidad. La cirugía logró márgenes microscópicos libres, con una adecuada funcionalidad y sin recaída a los 42 meses de seguimiento.

Abstract The patients with soft tissue sarcomas of the extremities are best treated with wide local excisions, with resection of the primary tumor and normal tissue around the lesion, in order to achieve negative margins; usually when the nerves, the vessels or the bone are surrounded by the tumor, amputation is needed. We report the case of a patient with Liposarcoma in the popliteal fossa, with the tumor involving the nerve and popliteal vessels. The patient underwent to limb-sparing treatment, including preoperative radiotherapy, wide local excision and intraoperative radiotherapy, allowing to keep the extremity, with negative margins, acceptable function of the limb and without recurrence after 42 months of follow up.

Hamartoma fibroso de la infancia umbilical: reporte de un caso / Umbilical fibrous hamartoma of infancy: a case report

Resumen: Introducción: el hamartoma fibroso de la infancia (HFI) es un tumor benigno de partes blandas que se presenta generalmente en población infantil y que posee una morfología histológica característica. Objetivo: describir un caso de HFI congénito de características clínicas e histológicas atípicas. Caso Clínico: recién nacido de término, sexo masculino, sin antecedentes mórbidos perinatales, es deriva do a dermatología por placa eritematosa congénita en región umbilical. Estudio histológico evidenció proliferación fusocelular en dermis e hipodermis, de morfología bifásica, con un patrón arremolina do infiltrante y otro de bandas de células fusadas con hábitos fibroblásticos y miofibroblásticos, aso ciada en profundidad a un componente de tejido adiposo maduro. El estudio inmunohistoquímico mostró positividad difusa a CD34 y focalmente para FXIIIa, con ausencia de inmunoreactividad a ac- tina, desmina, MyoD1, S100, HMB45, Melan A y EMA. Fluorescent in situ hybridization (FISH) para platelet-derived growth factor beta (PDGF beta) y para el gen ETV6 negativos, presentes en el dermatofibrosarcoma protuberans congénito y fibrosarcoma infantil, respectivamente. Estos antecedentes, sumado a los hallazgos histológicos previos, apoyaron el diagnóstico de HFI. Se realizó extirpación quirúrgica, sin signos de recidiva durante el seguimiento clínico. Conclusión: es importante consi derar el HFI dentro del diagnóstico diferencial de tumores subcutáneos infantiles, principalmente en población menor de 2 años. Si bien su comportamiento es benigno, presenta similitud con múltiples lesiones benignas y malignas, lo que hace imperativo realizar un estudio histológico exhaustivo ante lesiones clínicas sospechosas.

Abstract: Introduction: Fibrous hamartoma of infancy (FHI) is a benign, soft tissue tumor that usually oc curs in children and has a characteristic histological morphology. Objective: To describe a case of congenital FHI with atypical histological and clinical characteristics. Clinical case: Full-term male newborn, with no perinatal morbid history was referred to dermatology due to a congenital erythe matous plaque in the umbilical region. The histological study showed a fusocelullar proliferation in dermis and hypodermis of biphasic distribution, with an infiltrative, swirling pattern and bundles of spindle fibroblast-like and myofibroblast-like cells, associated in depth with a mature adipose tissue component. The immunohistochemical study revealed diffuse positivity for CD34, and focal posi tivity for FXIIIa, without immunoreactivity for actin, desmin, MyoD1, S100, HMB45, Melan-A, or EMA. Fluorescent in situ hybridization (FISH) was negative for platelet-derived growth factor recep tor beta (PDGFR-beta) and for ETV6 gene. PDGFR-beta and ETV6 gene are present in congenital dermatofibrosarcoma protuberans and infantile fibrosarcoma, respectively. This history, in addition to previous histological findings, supported the diagnosis of FHI. Surgical resection was performed, without signs of recurrence during clinical follow-up. Conclusion: It is important to consider the FHI within the differential diagnosis of subcutaneous tumors in children, especially in those under two years of age. Although its behavior is benign, it is similar to multiple benign and malignant le sions, which makes it imperative to perform a histological study in front of suspicious clinical lesions.

Myopericytoma - A Unifying Term Or A Unique Entity?

Myopericytoma are uncommon, slow-growing benign perivascular neoplasms that show hemangiopericytoma-like vascular pattern. We report a 52-year-male patient with a painless palpable nodule in the left thumb for the past 9 months. The mass, on the thenar aspect, was 15x12mm in size. X-ray revealed a soft tissue swelling with no bony association. The excised nodule was a non-capsulated, well-circumscribed vascular neoplasm composed of proliferating spindle to ovoid bland cells with eosinophilic cytoplasm. A concentric perivascular arrangement of the cells was seen interspersed by thin-walled, branching, staghorn blood vessels. Nuclear atypia, mitotic figures and necrosis were not observed. Immunohistochemistry revealed diffuse positivity of the tumour cells for smooth muscle actin while staining negative for CD34 and desmin - features suggestive of origin from the perivascular myoid cell. Morphological features of myopericytoma are shared with hemangiopericytoma, glomus tumors, myofibroma and solitary fibrous tumour which form the important differential diagnoses. It is a relatively newly described disease entity recognized by the World Health Organisation classification of tumours.

Giant labial fi broepithelial stromal polyp

We report an18-year-old girl with a four-year history of a slow-growing labial mass with a sudden increase in size in the last year. Examination revealed a large fl eshy 20 cm perineal mass centering on the left labia majora and attached to it by a 1cm pedicle. It was associated with pain, ulceration and discharge. The lesion was excised via diathermy at the base of the stalk. The excised specimen weighed 1.112kg and measured 20.5 x 17 x 5cm. The lesion showed a solid, soft whitish, cut surface. Histology revealed a hypocellular tumour with focally oedematous fi brous stroma in which were scattered large and small blood vessels, mast cells and other chronic infl ammatory cells. True myxoid matrix was not observed. The stromal cells had a spindle to stellate morphology. There was no signifi cant cytological atypia, mitotic activity or necrosis. The tumour cells were negative for SMA, desmin, CD34, S100 protein, EMA and PR. The diagnosis was clinically and histologically challenging because various vulvovaginal soft tissue tumours often have overlapping clinicopathological features. However, based on strict histological criteria and the absence of worrisome cytological features, a diagnosis of fi broepithelial stromal polyp was rendered despite the unusual size. A review of the literature shows that whilst vulvovaginal fi broepithelial stromal polyps are well described, giant variants are rare. Awareness of the extraordinary size that can be attained by such polyps can fascilitate swift clinical and histological diagnosis.

A fifty-year review of soft tissue sarcomas in Jamaica: 1958-2007 / Cincuenta años de revisión de los sarcomas de tejidos blandos en Jamaica: 1958-2007

OBJECTIVE: To determine the distribution of histologic subtypes of soft tissue sarcomas (STS) in Kingston and St Andrew, Jamaica, according to age and topography. METHODS: From the Jamaica Cancer Registry (JCR) archives, all cases of STS diagnosed between 1958 and 2007 were extracted. For each case, age, gender, histological diagnosis and anatomical site of tumour were recorded. Patients were categorized according to age at diagnosis as: children (0-14 years) and adults (> 14 years), and the distribution of histologic diagnoses with respect to age and anatomical site were analysed. RESULTS: There were 432 cases (67 children, 364 adults, one person of unknown age) of STS recorded in the JCR over the 50-year period (218 males, 214 females). The commonest STS in adults were "sarcoma, not otherwise specified [NOS]" (20.1%), malignant fibrous histiocytoma [MFH] (17.9%), fibrosarcoma (12.4%), liposarcoma (10.7%) and malignant peripheral nerve sheath tumour [MPNST] (10.2%). In children, they were neuroblastoma (38.8%), rhabdomyosarcoma (23.9%), "sarcoma, NOS" (9%), fibrosarcoma (6%) and MFH (6%). In adults, the lower limb was the commonest location, followed by trunk and/or upper limb for MFH, fibrosarcoma and liposarcoma, and head and neck for MPNST. In children, head and neck was the commonest site for rhabdomyosarcoma, head and neck and upper limb for MFH, retroperitoneum for neuroblastoma and trunk for fibrosarcoma. CONCLUSION: A high proportion of soft tissue sarcomas in Jamaica are unclassified and the anatomical distribution of common classified sarcomas shows some differences with the literature. Limited access to immunohistochemistry/molecular diagnostics and increasing core biopsy diagnosis may contribute to these phenomena.

OBJETIVO: Determinar la distribución de subtipos histológicos de sarcomas de tejido blando (STB) en Kingston y Saint Andrew, Jamaica, según la edad y la topografía. MÉTODOS: De los archivos del Registro de Cáncer de Jamaica, se extrajeron todos los casos de STB diagnosticados entre 1958 y 2007. Para cada uno de los casos, se registró la edad, el género, el diagnóstico histológico, y el sitio anatómico del tumor. Los pacientes fueron clasificados de acuerdo con la edad en el momento del diagnóstico, bajo las categorías de niños (0-14 años) y adultos (> 14 años), y se analizó la distribución de diagnósticos histológicos con respecto a la edad y el sitio anatómico. RESULTADOS: Se registraron 432 casos de STB (67 niños, 364 adultos, una persona de edad desconocida) en el JCR en un período de 50 años (218 varones, 214 hembras). Los STB más comunes en los adultos fueron "el sarcoma no especificado [NE]" (20.1%), el histiocitoma fibroso maligno [HFM] (17.9%), el fibrosarcoma (12.4%), el liposarcoma (10.7%), y el tumor maligno de la vaina del nervio periférico [TMVNP] (10.2%). En los niños, se trató de los neuroblastomas (38.8%), los rabdomiosarcomas (23.9%), "los sarcomas NE" (9%), los fibrosarcomas (6%), y los HFM (6%). En los adultos, los miembros inferiores fueron el lugar más común, seguido del tronco y/o los miembros superiores para el HFM, el fibrosarcoma y el liposarcoma; y la cabeza y el cuello para el TMVNP. En los niños, la cabeza y el cuello fueron el sitio más común para el rabdomiosarcoma; la cabeza, el cuello y los miembros superiores para el HFM; el retroperitoneo para el neuroblastoma; y el tronco para el fibrosarcoma. CONCLUSIÓN: Una proporción alta de sarcomas de tejidos blandos en Jamaica no están clasificados, y la distribución anatómica de sarcomas clasificados comunes muestran algunas diferencias con la literatura. El acceso limitado a los diagnósticos moleculares/inmunohistoquímicos, y el aumento de los diagnósticos centrales, pueden contribuir a estos fenómenos.

Kimura Disease: An Unusual Presentation in Paediatric Age Group

Kimura disease is a chronic inflammatory disorder that is prevalent among Asians. It rarely affects the upper limbs or paediatric patients. We report a case of Kimura disease with first presentation as a soft tissue mass in the arm mimicking a soft tissue neoplasm with metastasis in a 12 year old Chinese boy.

Clear Cell Sarcoma of the Knee. A Report of Two Rare Cases

Clear cell sarcoma of soft tissue is a rare type of soft tissue sarcoma. It is derived from melanoblast like cells located within subcutaneous tissue, tendon and aponeuroses. The tumour is also known as malignant melanoma of soft parts because it has similar morphology to malignant melanoma. Unlike malignant melanoma, however, it is not associated with a cutaneous lesion. We report here two cases of this tumour occurring in young adults.