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Jin Wang - One of the best experts on this subject based on the ideXlab platform.

  • Infant pleuropulmonary blastoma: report of a rare case and review of literature.
    International journal of clinical and experimental pathology, 2015
    Co-Authors: Hong Zhang, Jian-guo Wei, Guang-jun Zhu, Jin Wang
    Abstract:

    In infants, pleuropulmonary blastoma is a rare but aggressive tumor. The typical histopathological presentation includes the aggregation of malignant primitive small cells, usually observed in sheets. So as to provide proper and timely treatment, the differential diagnosis includes pulmonary blastoma, sarcomatoid mesothelioma, Fetal Rhabdomyoma, synovial sarcoma, and primitive neuroectodermal tumor. Herein, we will present one male pediatric patient with pleuropulmonary blastoma. The patient was a 4-month-old male infant, who had a prolonged cough and dyspnea for 4 months that was complicated by cyanosis for 3 days. A physical examination revealed a solid mass in the right lung that was sized 9.0 × 6.0 × 4.0 cm and had a grayish-white cross section. The boundary between the mass and lung tissue was clear; the mass already occupied a great portion of the lung. A microscopic examination suggested that the tumor was composed of round or orbicular-ovate primitive Fetal cells. The cells were medium sized, having little cytoplasm, but had a clearly visualized nucleolus and active karyokinesis. The tumor mass was biphasic, namely, fasciculated sarcoma (composed of spindle-shaped cells and short spindle-shaped cells) and malignant fibrous histiocytoma containing well-differentiated cartilage islands or cartilaginous nodes. Immunohistochemistry was performed for further detection: vimentin (+), S-100 protein (+), CK (AE1/AE3), EMA and TTF-1 in residual epithelial components (+), NSE (focal +), SMA (mesenchymal cells, focal +), CD99 (weak +), Bcl-2 (weak +), desmin (-), myoglobin (-), calretinin (-), calponin (-), FLI (-), MyoD-1 (-), and CD34 (-). Pleuropulmonary blastoma is extremely rare but highly aggressive neoplasm in children. Its typical histopathological presentation is the aggregation of primitive malignant small cells. Combining imaging and histopathological examinations and clinical data should help in determining the diagnosis of pleural pulmonary blastoma.

  • Case Report Infant pleuropulmonary blastoma: report of a rare case and review of literature
    2015
    Co-Authors: Hong Zhang, Jian-guo Wei, Guang-jun Zhu, Jin Wang
    Abstract:

    In infants, pleuropulmonary blastoma is a rare but aggressive tumor. The typical histopathological pre- sentation includes the aggregation of malignant primitive small cells, usually observed in sheets. So as to provide proper and timely treatment, the differential diagnosis includes pulmonary blastoma, sarcomatoid mesothelioma, Fetal Rhabdomyoma, synovial sarcoma, and primitive neuroectodermal tumor. Herein, we will present one male pediatric patient with pleuropulmonary blastoma. The patient was a 4-month-old male infant, who had a prolonged cough and dyspnea for 4 months that was complicated by cyanosis for 3 days. A physical examination revealed a solid mass in the right lung that was sized 9.0 × 6.0 × 4.0 cm and had a grayish-white cross section. The boundary between the mass and lung tissue was clear; the mass already occupied a great portion of the lung. A microscopic examination suggested that the tumor was composed of round or orbicular-ovate primitive Fetal cells. The cells were medium sized, having little cytoplasm, but had a clearly visualized nucleolus and active karyokinesis. The tumor mass was biphasic, namely, fasciculated sarcoma (composed of spindle-shaped cells and short spindle-shaped cells) and malignant fibrous histiocytoma containing well-differentiated cartilage islands or cartilaginous nodes. Immunohistochemistry was performed for further detection: vimentin (+), S-100 protein (+), CK (AE1/AE3), EMA and TTF-1 in residual epithelial components (+), NSE (focal +), SMA (mesenchymal cells, focal +), CD99 (weak +), Bcl-2 (weak +), desmin (-), myoglobin (-), calretinin (-), calponin (-), FLI (-), MyoD-1 (-), and CD34 (-). Pleuropulmonary blastoma is extremely rare but highly aggressive neoplasm in children. Its typical histopathological presentation is the aggregation of primitive malignant small cells. Combining imaging and histopathological examinations and clini- cal data should help in determining the diagnosis of pleural pulmonary blastoma.

F. Contreras - One of the best experts on this subject based on the ideXlab platform.

  • Neural variant of Fetal Rhabdomyoma and naevoid basal cell carcinoma syndrome.
    Histopathology, 1996
    Co-Authors: David Hardisson, José A. Jiménez-heffernan, Manuel Nistal, M.l. Picazo, J.a. Tovar, F. Contreras
    Abstract:

    A 15-year-old boy with the characteristic features of the naevoid basal cell carcinoma syndrome presented with a retroperitoneal mass. The tumour showed morphological features of a still ill-defined variant of Fetal Rhabdomyoma, characterized by well-differentiated nerve fibres admixed with immature striated muscle cells, similar to neuromuscular choristoma. Four cases of Fetal Rhabdomyoma and naevoid basal cell carcinoma syndrome have been previously reported. The behaviour of this tumour has been benign, although a complete excision was impossible due to its close relation with abdominal vascular trunks.

Pauline M Chou - One of the best experts on this subject based on the ideXlab platform.

  • Spindle cell rhabdomyosarcoma of the tongue in an infant: a case report with emphasis on differential diagnosis of childhood spindle cell lesions.
    Pathology research and practice, 2004
    Co-Authors: Anita Gupta, John Maddalozzo, Thanda Win Htin, Anil Shah, Pauline M Chou
    Abstract:

    Malignant tumors are extremely uncommon in infants, specifically in the head and neck region. We present a three-day-old infant with a large, polypoid, soft tissue mass arising from the floor of the mouth. Histologically, this neoplasm consisted of hypercellular and myxoid areas. A mixture of poorly oriented, small, undifferentiated, hyperchromatic, and round to elongate spindle cells was seen. A high degree of striated muscle differentiation was present, along with areas marked by a herringbone pattern, as well as hemangiopericytic vessels and rare mitosis. Immunohistochemical examinations revealed strong nuclear staining for myogenin and diffuse cytoplasmic staining for desmin and muscle-specific actin (HHF-35). The tumor did not stain for S-100. Based on histologic results and immunostains, this lesion was diagnosed as spindle cell rhabdomyosarcoma. This type of lesion involving the tongue is rarely seen in females, neither in association with a herringbone pattern nor with hemangiopericytic vessels. Furthermore, rare benign and malignant spindle lesions, such as cellular fibromatosis, Fetal Rhabdomyoma, infantile hemangiopericytoma, infantile rhabdomyofibrosarcoma, and infantile fibrosarcoma, should be in the differential diagnosis and excluded.

Hong Zhang - One of the best experts on this subject based on the ideXlab platform.

  • Infant pleuropulmonary blastoma: report of a rare case and review of literature.
    International journal of clinical and experimental pathology, 2015
    Co-Authors: Hong Zhang, Jian-guo Wei, Guang-jun Zhu, Jin Wang
    Abstract:

    In infants, pleuropulmonary blastoma is a rare but aggressive tumor. The typical histopathological presentation includes the aggregation of malignant primitive small cells, usually observed in sheets. So as to provide proper and timely treatment, the differential diagnosis includes pulmonary blastoma, sarcomatoid mesothelioma, Fetal Rhabdomyoma, synovial sarcoma, and primitive neuroectodermal tumor. Herein, we will present one male pediatric patient with pleuropulmonary blastoma. The patient was a 4-month-old male infant, who had a prolonged cough and dyspnea for 4 months that was complicated by cyanosis for 3 days. A physical examination revealed a solid mass in the right lung that was sized 9.0 × 6.0 × 4.0 cm and had a grayish-white cross section. The boundary between the mass and lung tissue was clear; the mass already occupied a great portion of the lung. A microscopic examination suggested that the tumor was composed of round or orbicular-ovate primitive Fetal cells. The cells were medium sized, having little cytoplasm, but had a clearly visualized nucleolus and active karyokinesis. The tumor mass was biphasic, namely, fasciculated sarcoma (composed of spindle-shaped cells and short spindle-shaped cells) and malignant fibrous histiocytoma containing well-differentiated cartilage islands or cartilaginous nodes. Immunohistochemistry was performed for further detection: vimentin (+), S-100 protein (+), CK (AE1/AE3), EMA and TTF-1 in residual epithelial components (+), NSE (focal +), SMA (mesenchymal cells, focal +), CD99 (weak +), Bcl-2 (weak +), desmin (-), myoglobin (-), calretinin (-), calponin (-), FLI (-), MyoD-1 (-), and CD34 (-). Pleuropulmonary blastoma is extremely rare but highly aggressive neoplasm in children. Its typical histopathological presentation is the aggregation of primitive malignant small cells. Combining imaging and histopathological examinations and clinical data should help in determining the diagnosis of pleural pulmonary blastoma.

  • Case Report Infant pleuropulmonary blastoma: report of a rare case and review of literature
    2015
    Co-Authors: Hong Zhang, Jian-guo Wei, Guang-jun Zhu, Jin Wang
    Abstract:

    In infants, pleuropulmonary blastoma is a rare but aggressive tumor. The typical histopathological pre- sentation includes the aggregation of malignant primitive small cells, usually observed in sheets. So as to provide proper and timely treatment, the differential diagnosis includes pulmonary blastoma, sarcomatoid mesothelioma, Fetal Rhabdomyoma, synovial sarcoma, and primitive neuroectodermal tumor. Herein, we will present one male pediatric patient with pleuropulmonary blastoma. The patient was a 4-month-old male infant, who had a prolonged cough and dyspnea for 4 months that was complicated by cyanosis for 3 days. A physical examination revealed a solid mass in the right lung that was sized 9.0 × 6.0 × 4.0 cm and had a grayish-white cross section. The boundary between the mass and lung tissue was clear; the mass already occupied a great portion of the lung. A microscopic examination suggested that the tumor was composed of round or orbicular-ovate primitive Fetal cells. The cells were medium sized, having little cytoplasm, but had a clearly visualized nucleolus and active karyokinesis. The tumor mass was biphasic, namely, fasciculated sarcoma (composed of spindle-shaped cells and short spindle-shaped cells) and malignant fibrous histiocytoma containing well-differentiated cartilage islands or cartilaginous nodes. Immunohistochemistry was performed for further detection: vimentin (+), S-100 protein (+), CK (AE1/AE3), EMA and TTF-1 in residual epithelial components (+), NSE (focal +), SMA (mesenchymal cells, focal +), CD99 (weak +), Bcl-2 (weak +), desmin (-), myoglobin (-), calretinin (-), calponin (-), FLI (-), MyoD-1 (-), and CD34 (-). Pleuropulmonary blastoma is extremely rare but highly aggressive neoplasm in children. Its typical histopathological presentation is the aggregation of primitive malignant small cells. Combining imaging and histopathological examinations and clini- cal data should help in determining the diagnosis of pleural pulmonary blastoma.

Lisa K. Hornberger - One of the best experts on this subject based on the ideXlab platform.

  • Fetal Rhabdomyoma: prenatal diagnosis, clinical outcome, and incidence of associated tuberous sclerosis complex
    The Journal of pediatrics, 2003
    Co-Authors: Rima S. Bader, David Chitayat, Edmond Kelly, Greg Ryan, Jeffrey F. Smallhorn, Ants Toi, Lisa K. Hornberger
    Abstract:

    Abstract Objectives We reviewed our institution's experience with Fetal cardiac Rhabdomyoma to document the clinical outcome and incidence of associated tuberous sclerosis complex (TSC) and compared our findings with those of patients diagnosed with cardiac Rhabdomyoma after birth. Study design We reviewed the medical records of all cases diagnosed prenatally and postnatally with cardiac Rhabdomyoma between January 1990 and June 2002. Results Twenty fetuses with cardiac Rhabdomyoma were diagnosed at 28.4±6.0 weeks' gestational age. Of 19 continued pregnancies, there was one spontaneous intrauterine death, and 18 were delivered at term. Although none had prenatal hemodynamic complications, after birth seven had cardiac symptoms requiring medical (n = 4) or surgical intervention (n = 3). On follow-up, 15 of 19 with available outcome had TSC (79%), including six with neurodevelopmental disease. Over the same period, 26 patients were diagnosed with cardiac Rhabdomyoma postnatally. Most (77%) were referred for cardiac assessment after findings suggesting TSC. On follow-up, TSC was confirmed in 25 (96%), including 22 with neurodevelopmental disease. The incidence of cardiac symptoms and TSC was not statistically different between the prenatal and postnatal diagnosis groups. Conclusions Cardiac Rhabdomyomas are benign from the cardiovascular standpoint in most affected fetuses. As observed in postnatally diagnosed cardiac Rhabdomyoma, TSC is diagnosed in most cases of Fetal cardiac Rhabdomyoma.