The cells are small, round, large round or ovoid nuclei, dispersed chromatin, scanty. Small blue round cell tumor an overview sciencedirect. Small round cell neoplasms, a subset of childhood malignancies, are his tologically similar but. Everyday practice may occasionally be more challenging. Learn more about how this cancer forms, is treated, and the prognosis. Small round cell tumor of the head and neck region. Small round blue cell tumor with bcorccnb3translocation pierrone et al. Because of their primitive histologic appearance many childhood tumor have been collectively referred to as small round blue cell tumor. These include ewings sarcoma, peripheral neuroectodermal tumors, rhabdomyosarcoma, synovial sarcoma, nonhodgkins lymphoma, retinoblastoma. These cells at low magnification resemble mature lymphocytes which also posses scant cytoplasm. Immunohistochemical stains were performed to differentiate and categorise the small round blue cell tumours. A definitive diagnosis could not be fully made, even with immunohistochemical and genetic testing.
Introduction small round cell tumor is the traditional generic name given to a group of undifferentiated tumors occurring with predilection in children and young adults in which light microscopy alone is not. Small round blue cell tumors srbcts are characterized as sheets of cells with small, hyperchromatic round nuclei and scanty cytoplasm and represent a diverse group of tumors which pose a number of problems in diagnosis. Diagnosis of the small round blue cell tumors using. Pdf the small round blue cell tumors of the sinonasal area. Histologically, many of the pediatric neoplasms have more primitive origin characterized by sheets of cells,with small, round nuclei. Bone marrow infiltration in small blue cell tumours. These tumors share several overlapping histologic and immunophenotypic findings while also showing considerable variation within and between cases.
Rhabdomyosarcoma in a four year old child, pakistan. The differential diagnosis of sinonasal small round blue cell tumors and tumors with neuroendocrine differentiation at this site is broad and complex 6, 21,25,34,36,43,47,59, including. These tumors all show similar histologic features of densely packed, small, round blue cells withoutdistinctcellborders. Small round blue cell tumor of seminal vesicle in a. Small cell tumors of bone md anderson cancer center. Jul 01, 2011 small blue round cell tumors of childhood rarely present in the foot or ankle. One of the most challenging diagnostic categories within tumors of the sinonasal tract is the small round blue cell tumors. Reported sites of metastatic spread include the liver, lungs, lymph. Small round blue cell tumors srbct is a group of neoplasms that share a common and unique chromosomal transloca tion and include the primitive. These include ewings sarcoma, peripheral neuroectodermal tumors, rhabdomyosarcoma.
Dsrct is very rare and most commonly occurs in white males between the ages of 10 and 30 years old. Biopsies are usually small and limited, resulting in considerable diagnostic difficulty for practicing surgical pathologists. The small round cell tumor srct family is a diverse group of neoplasms that are primitive or embryonal in. In histopathology, a small blue round cell tumour, also known as a small round blue cell tumor or a small round cell tumour, is any one of a group of malignant neoplasms that have a characteristic appearance under the microscope, i. However are significantly larger than small lymphocytes and erythrocytes. Small blue round cell tumor an overview sciencedirect topics. Mar 29, 2021 the small round blue cell tumors include neuroblastoma, rhabdomyosarcoma, nonhodgkins lymphoma, and the ewings family of tumors. Mar 01, 2016 in a textbook scenario, any of the small, round, blue cell tumors srbct of the sinonasal tract have classical radiologic features and key histologic findings that aid in their distinction. Small round blue cell tumors radiology reference article. The predominance of blue staining is because the cells consist predominant. Small round blue cell tumor srbct or a small round cell tumour srct is a group of malignant neoplasms which are seen more often in children 020 yearsold than in adults.
Malignant small round cell tumors is the name given to a group of malignant neoplasms that occur mostly in the pediatric age group up to 14 yrs. Small blue round cell tumor journal of the american. Smallcell carcinoma of the ovary, hypercalcemic type. Diagnostic utility of mic2 immunocytochemical staining in the differential diagnosis of small blue cell tumors. The following is a case presentation of an 18yearold male with a large softtissue mass of the foot with associated lung metastasis. Definition group of highly aggressive malignant neoplasm which are composed predominantly of small, round and monotonous undifferentiated cells with high nuclearcytoplasmic ratio. The small round blue cell tumors, and especially sarcomas, are a challenging and diverse group of tumor types. As nucleus stains blue and cytoplasm pink, these tumors impart a distinct blue appearance.
Highly aggressive undifferentiated small round blue cell. This classification is based on a variety of publications showing that each of these molecular subtypes has unique clinical and prognostic characteristics distinct from ewing sarcoma, therefore. Reported sites of metastatic spread include the liver, lungs, lymph nodes, brain, skull, and bones. Differential diagnosis of paediatric small round and blue cell tumors an update ivo leuschner kiel paediatric tumor registry dept. The correct diagnosis of small cell tumors of bone depends on an evaluation. The new 5th edition of the who classification of tumors of soft tissue and bone recognizes four subgroups of undifferentiated round cell sarcomas. Immunohistochemical characterization of small round blue cell. Malignant small round cell tumors small blue cell malignancies prototypically are composed of cells with round to oval nuclei, scant cytoplasm, and often extensive cellular dissociation. Soft tissue small round blue cell tumors of childhood. Malignancies characterized histologically by highgrade monotonous small round blue cells srbcs belong to a heterogeneous group of neoplasms often referred to as ewing family of tumors.
Diagnostic utility of mic 2 immunocytochemical staining in the differential diagnosis of small blue ce. The group of small round blue cell tumors of childhood represents a descriptive category of malignant pediatric neoplasms characterized by the presence of morphologically poorly differentiated cells containing large hyperchromatic nuclei and scant cytoplasm. Based on histopathological patterns ewings sarcoma they are composed of solid sheets or masses of small round cells with little stroma, few connective tissue septa may be present. Small round blue cell tumors of the sinonasal tract. Sccoht is the prototypical ovarian neoplasm composed predominantly or exclusively of small round cells with scant cytoplasm socalled small round blue cell tumor. Desmoplastic small round cell tumors dsrct are tumors that grow in the abdomen and pelvic area of the body. The tumor has a specific genetic abnormality that helps confirm the diagnosis. Differential diagnosis of paediatric small round and blue. This article beautifully illustrations several of the round cell carcinomas using ewing versus cicrearrangement studies. Ewing sarcoma, cicrearranged sarcomas, bcoraltered sarcomas, and sarcomas with ewsr1nonets fusions, in addition to desmoplastic small round cell tumor. The cytoplasm of these cells is pale blue and contains variable numbers of. Other areas affected may include the lymph nodes, the lining of the abdomen, diaphragm, spleen, liver, chest wall, skull, spinal cord, large intestine, small intestine, bladder, brain, lungs, testicles, ovaries, and the pelvis. Accurate diagnosis of these cancers, which include neuroblastoma nb, rhabdomyosarcoma rms, nonhodgkins lymphoma, and the ewings family of tumors ews, is essential because.
Medulloblastoma melanoma rhabdomyosarcoma retinoblastoma. It encompasses a wide variety of undifferentiated primitive neoplasms which have in common the ability, with varying frequency, to present as a nondescript proliferation of round cells with high nuclear cytoplasmic ratio and hyperchromatic nuclei by. A tenyear retrospective research of the histopathological and immunohistochemical features of small round blue cell tumours srbct. Small round cell tumor diagnostic approach dr niharika singh md pathology ii yearresident gandhi medical college. Today, srbcts are diagnosed with the use of multiple parameters of which immunohistochemistry plays an important role.
Diagnosis of the small round blue cell tumors using multiplex. Small round blue cell sarcoma other than ewing sarcoma. A number of tumors share similar histology, composed of relatively uniform primitive small round blue cells. This group of tumors encompasses a wide diversity of both benign and malignant neoplasms. At the same time, a free text of manuscript is available in html and pdf format. These tumors are seen more often in children than in adults. In any case, small round blue cell tumors are malignant tumors that are hard to distinguish from each other using conventional microscopic analysis. The sinonasal small round blue cell tumor srbct differential diagnosis is extensive and fraught with pitfalls, necessitating a thoughtful immunohistochemical. Desmoplastic small round cell tumors dsrct national. The small round blue cell tumors are a group of neoplasms occurring usually in children and adolescents and are characterized by small round relatively undifferentiated cells. The correct diagnosis of small cell tumors of bone depends on an evaluation of clinical, radiologic, pathologic, and genetic features. Pdf the diagnostic classification of small round blue cell tumors of the sinonasal area to include diverse malignancies of epithelial. An organoid growth pattern is also potentially apparent figs.
Small round blue cell tumors of the sinonasal tract lester dr. The most common molecular confirmation of these neoplasms is by fusions between ewsr1 gene on chromosome 22 and the ets family of transcription factors, including fli1 gene 11q24 and the erg 21q22, that are implicated in the development of different tissues as well as cancer progression. Immunohistology, cytogenetics, and molecular studies of small. Small round blue cell tumor with cicdux4translocation antonescu, 20 cd99. In addition, other small, blue, round cell tumors typically occurring in soft tissue, including neuroblastoma and rhabdomyosarcoma, frequently relapse in bone in the.
There is fast growing authorship and readership with jcdr as this can be judged by the number of articles published in it i e. Because of the wide range of differential diagnoses of the various neoplasms in this broad group, pathologists commonly struggle with these tumors due to overlapping. Immunohistochemical characterization of small round blue. One such area is the small round blue cell tumor category. Pdf thoracic presentations of small round blue cell tumors. Small round cell tumours, immunohistochemistry jcdr. Unfortunately, interpretation of stains in these tumors is fraught with numerous pitfalls and limitations. The disparity in treatment modalities, and hence, clinical outcome in the different subsets of sbrcts makes the correct diagnosis crucial.
The smallround cell tumor srct family is a diverse group of neoplasms that are primitive or embryonal in. Small cell lung cancer lymphoma esthesioneuroblastoma ewings sarcoma peripheral neuroectodermal tumor. Furthermore, metastatic small cell carcinoma in bone is not an. This article presents an approach to sinonasal small round blue cell tumors based on four common immunohistochemical patterns. Tumors are formed by small, round cancer cells and are surrounded by scarlike tissue. They also share many demographic, radiographic and clinical similarities. Desmoplastic smallroundcell tumor is an aggressive and rare cancer that primarily occurs as masses in the abdomen. Small round cell tumors configure a heterogeneous group of malignant neoplasms that present clinical predominance in children and young adults. Desmoplastic small round cell tumor dsrct is a type of cancer. Oct 19, 20 small round blue cell tumors srbcts mnemonic. Jcdr small round cell tumours, immunohistochemistry. In histopathology, a small blue cell tumor or small round blue cell tumors refer to tumors comprised of blue cell with very scant cytoplasm. Pdf small round blue cell tumors of the sinonasal tract. Sometimes other tumor types are included in this group.
Small round blue cell tumors lymphoma neuroblastoma rhabdomyosarcoma ewing sarcomaprimitive neuroectodermal tumor pnet desomplastic small round cell tumor poorly differententiated synovial sarcoma small cell osteosarcoma ddx. The primary pleuropulmonary lesions in this category include neuroendocrine carcinomas, malignant lymphomas, and very rare malignancies, such as askin tumor or small. Although each entity has its distinctive features, the differential diagnosis of this group of tumors is still challenging because they are all small, blue, and round cell tumors, histologically. Malignant tumor of neuroectodermal differentiation that is often arises in the bone but can see in many organs. The highly malignant small round blue cell tumors srbcts occur in the pediatric, adolescent, and young adult populations in some cases. Pdf immunohistochemical characterization of small round blue. It is usually found in the abdomen belly, but it can also occur in other parts of the body. Pdf the term small round blue cell is frequently used as a cursory radiologic pathological correlation of aggressive tumors throughout the. These tumors show characteristic appearance under the mi croscope, consist of small round cells that stain blue on routine haematoxylin and eosin stain.
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