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Diagnosis and Differential Diagnosis of Desmoplastic Fibroblastoma by Clinical, Radiological, and Histopathological Analyses

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Chinese Medical Journal 2018年第1期131卷 32-36页

作者： Li-Hua Gong Wei-Feng Liu Yi Ding Yue-Hang Geng Xiao-Qi Sun Xiao-Yuan Huang Department of Pathology Beijing Jishuitan Hospital The Fourth Medical College of Peking University Beijing 100035 China Department of Orthopedic Oncology Beijing Jishuitan Hospital The Fourth Medical-College of Peking University Beijing 100035 China Department of Pathology Peking University Health Science Center Beijing 100191 China

Background： Desmoplastic fibroblastoma （collagenous fibroma） is an uncommon benign soft-tissue tumor, rarely involving bone. It shares some overlapping features with other infiltrate tumors, such as desmoid-type fibromatosis, neurofibroma, and low-grade fibromyxoid sarcoma. The misdiagnosis may cause unnecessary surgical overtreatment, especially for those involving bone. In order to deepen the understanding of the diagnosis and differential diagnosis of Desmoplastic fibroblastoma, we planned to analyze the clinical, radiological, and histopathological features and the outcome of Desmoplastic fibroblastoma on the basis of case analysis and literature review. Methods： Sixteen cases were retrieved from the surgical pathology records from May 2011 to April 2016 in the Department of Pathology in Beijing Jishuitan Hospital. Formalin-fixed, paraffin-embedded specimens of 16 cases of Desmoplastic fibroblastoma were collected. Hematoxylin and eosin stain and immunohistochemistry were used to observe the histological features of Desmoplastic fibroblastoma of soft tissue and bone. The images for diagnosis obtained from the ultrasonic examination, X-ray, magnetic resonance imaging, and computed tomography were used to observe the radiological features. Related literatures were retrieved from the PubMed and CNKI databases. Results： Sixteen cases of Desmoplastic fibroblastoma of soft tissue were located in the hand （n = 7）, loot （n = 4）, upper arm （n = 1）, shoulder （n = 1）, forearm （n = 2）, and one case occurred in the proximal femur. Age ranged from 32 to 82 years （median age： 58 years）. There were six females and ten males. Histologically, the lesions of soft tissue appeared as well-circumscribed masses with abundant collagenous matrix and low vascularity. Tumor cells were stellate- or spindle-shaped and uniformly distributed within the extracellular matrix. In five cases, the Desmoplastic fibroblastoma were found to have infiltrated into the skeletal muscle tissue. In one case ofDesmoplastic fibroblastoma of bone, radiogra

关键词： Bone Collagenous Desmoplastic Diagnosis Differential Fibroma lmmunohistochemistry Soft Tissue

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Clinical and computed tomography features of adult abdominopelvic Desmoplastic small round cell tumor

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World Journal of Gastroenterology 2014年第17期20卷 5157-5164页

作者： Xun-Ze Shen Jian-Guo Zhao Jian-Jun Wu Fang Liu Department of Radiology Shaoxing People’s Hospital Shaoxing Hospital of Zhejiang University Department of Radiotherapy Shaoxing People’s Hospital Department of Radiology Second Affiliated Hospital of Medical SchoolZhejiang University Department of Pathology Shaoxing People’s Hospital

To investigate the clinical and computed tomography(CT)features of Desmoplastic small round cell tumor(DSRCT),we retrospectively analyzed the clinical presentations,treatment and outcome,as well as CT manifestations of four cases of DSRCT confirmed by surgery and pathology.The CT manifestations of DSRCT were as follows:(1)multiple soft-tissue masses or diffuse peritoneal thickening in the abdomen and pelvis,with the dominant mass usually located in the pelvic cavity;(2)masses without an apparent organbased primary site;(3)mild to moderate homogeneous or heterogeneous enhancement in solid area on enhanced CT;and(4)secondary manifestations,such as ascites,hepatic metastases,lymphadenopathy,hydronephrosis and hydroureter.The prognosis and overall survival rates were generally poor.Commonly used treatment strategies including aggressive tumor resection,polychemotherapy,and radiotherapy,showed various therapeutic effects.CT of DSRCT shows characteristic features that are helpful in diagnosis.Early discovery and complete resection,coupled with postoperative adjuvant chemotherapy,are important for prognosis of DSRCT.Whole abdominopelvic rather than locoregional radiotherapy is more effective for unresectable DSRCT.

关键词： Desmoplastic small round cell tumor Peritoneum Pat

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Desmoplastic small round cell tumor with atypical immunohistochemical profile and rhabdoid-like differentiation

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World Journal of Clinical Cases 2014年第8期2卷 367-372页

作者： Li Liang Nina Tatevian Meenakshi Bhattacharjee Kuojen Tsao John Hicks Department of Pathology and Laboratory Medicine the University of Texas Health Science Center at HoustonHoustonTX 77030United States Department of Pediatric Surgery the University of Texas Health Science Center at HoustonHoustonTX 77030United States Department of Pathology Texas Children’s HospitalHoustonTX 77030United States

Desmoplastic small round cell tumor(DSRCT) is a rare,aggressive malignant neoplasm of unknown origin, and is comprised of small round cells with a characteristic Desmoplastic stroma. DSRCT typically expresses epithelial, mesenchymal and neural markers simultaneously. We describe a case of DSRCT with an atypical immunohistochemical profile and rhabdoid-like tumor cells on electron microscopy. In the present case, the neoplastic cells were positive only for vimentin, desmin(cytoplasmic membranous pattern) and CD56,and negative for smooth muscle actin, synaptophysin,CD117, CD45, myogenin, CAM5.2, pancytokeratin,WT1, EMA, CD99, neurofilament, CD34 and p53. Ki67 showed a low proliferative activity. Electron microscopy showed focal rhabdoid differentiation. However, INI-1(SNF-5/BAF47) demonstrated preservation of nuclear positivity in the neoplastic cells. Cytogenetic studies showed translocation t(11;22)(p13;q12) confirming an EWSR1-WT1 translocation characteristic for DSRCT, and t(1;15)(q11;p11.2) of unknown significance. This case is a diagnostic challenge because of atypical immunohistochemical profile and cytogenetic study is crucial in rendering the correct diagnosis.

关键词： Desmoplastic small round cell tumor Ultrastructure Cytogenetics Rhabdoid cells EWSR1-WT1

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Ovarian Desmoplastic small round cell tumors: Prognosis is poor! Case report

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Open Journal of Obstetrics and Gynecology 2013年第2期3卷 235-238页

作者： Hanane Saadi Hind Fatmi Nissrine Mamouni Sanaa Errarhay Chahrazed Bouchikhi Afaf Amarti Abdelaziz Banani Department of Gynecology Obstetrics I University Hospital Hassan II Fez Morocco Department of Pathology University Hospital Hassan II Fez Morocco

The tumor Desmoplastic small round cell in women is rare. Ovarian its location is exceptional. We report a patient of 20 years, the originalNorth Africawho consulted for an increase in abdominal volume with impaired general condition. Pelvic ultrasonography and computed tomography were in favor of multiple peritoneal masses with ascites. After surgical exploration, the histological and immunohistochemical data for diagnosis small round cell Desmoplastic tumor of ovarian the patient was a candidate for poly chemotherapy but she was died a month later. Diagnosis relies on histological and immunohistochemical data. The demonstration of a nonrandom translocation t (11;22) (p13;q12) is specific for the disease. The management is multidisciplinary and combining surgery, a poly aggressive chemotherapy and radiotherapy. The prognosis remains poor.

关键词： Small Round Cell Desmoplastic Tumor Ovarian Diagnosis Treatment Prognosis

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Desmoplastic Ameloblastoma —A Case Report

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Open Journal of Stomatology 2017年第3期7卷 180-185页

作者： G. Gandhi A. Amirthagani Thanjavur Medical College and Hospital Thanjavur India

The Desmoplastic ameloblastoma (DA) is a rare variant of the conventional ameloblastoma. It accounts for only 4% to 13% of all ameloblastomas. Desmoplastic ameloblastoma (DA) was included in the World Health Organization Classification of Head and Neck Tumors (WHO-2005) as a variant of ameloblastoma with specific clinical, imaging and histological features. The Desmoplastic variant of ameloblastoma usually appears in the anterior and premolar regions as a mixed radiolucent and radiopaque lesion, sometimes resembling a benign fibro-osseous lesion [1]. Ameloblastoma is a locally aggressive tumor that may cause recurrence and in rare cases, malignant transformation with repeated postsurgical recurrences [2] [3]. In this paper we present a case of a 47-year-old female with swelling in the left upper jaw, bopsy of which turned out to be Desmoplastic ameloblastoma.

关键词： Ameloblastoma Desmoplastic Ameloblastoma Benign Fibro-Osseous Lesion Odontogenic Tumor

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Desmoplastic small round cell tumor of the abdomen: A case report and literature review of therapeutic options

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Health 2012年第4期4卷 207-211页

作者： Hafida Benhammane Leila Chbani Abdelmalek Ousadden Ouadii Mouquit Siham Tizniti Afaf Riffi Amarti Nouafal Mellas Omar El Mesbahi Department of general surgery Hassan II University Hospital Fez Morocco Department of medical oncology Hassan II University Hospital Fez Morocco Department of pathology Hassan II University Hospital Fez Morocco Department of radiology Hassan II University Hospital Fez Morocco

Desmoplastic small round cell tumor (DSRCT) is a rare and highly aggressive variety of sarcoma arising typically from abdominal or pelvic peritoneum. Diagnosis and treatment approaches of this entity are complex and require a skilled, experienced, multidisciplinary team. Authors report their experience with a case of an intraabdominal DSRCT arising in a 37-year-old young man in order to discuss the clinico-pathological and radiological behavior of this tumor and underline diagnostic and therapeutic difficulties.

关键词： Desmoplastic Small Round Cell Tumor Abdomen Histology Chemotherapy Surgery Outcome

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Hybrid Ameloblastoma in the Anterior Man-dible

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Advances in Computed Tomography 2015年第3期4卷 42-46页

作者： Mandana Pourian Mersad Mehranahad Pooneh Dehghan Radiology Department Rasool Akram Hospital Iran University of Medical Sci-ences Tehran Iran Taleghani Hospital Research Department Shahid Beheshti University of Med-ical Sciences Tehran Iran

Ameloblastoma—a benign epithelial odontogenic tumor-histologically occurs in different patterns, the most common of which are plexiform and follicular. “Hybrid” ameloblastoma is a variant in which areas of follicular or plexiform pattern (generally called convention alameloblastoma) coexist with areas of pronounced stromal desmoplasia (Desmoplastic ameloblastoma). They most often occur in the mandibular ramus. We present a case of “hybrid” lesion—Desmoplastic and conventional ameloblastoma—in the anterior mandible, focusing on its’ imaging features.

关键词： Odontogenic Tumor Hybrid Conventional Desmoplastic Ameloblastoma

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