Fibrous Tumor Research Articles

Management of Endolymphatic Sac Tumors

To analyze the difference between the endolymphatic sac tumors (ELSTs) in sporadic cases and in von Hippel-Lindau (VHL) disease. Retrospective case review in a tertiary referral center. Fourteen cases of ELST, occurring since 1998, were reviewed. We analyzed the initial symptoms, characteristics of the tumor, treatment, sequelae, and follow-up for each group. The ELSTs were sporadic in 6 cases and associated with VHL disease in 8 cases. The mean age at the time of the first surgery was 26 years (range, 12-41). All except two of the patients presented with a unilateral tumor. The initial symptoms were hearing loss (n = 9), tinnitus (n = 7), and/or vertigo (n = 5). Hearing loss was more prevalent in the sporadic cases. Preoperative arteriography was performed for 4 patients, with embolization performed for 1 patient. The size of the tumor was significantly larger in the sporadic cases (31.7 mm) than in the cases of VHL disease (19.3 mm). The surgical approach was more extensive in the sporadic cases. The surgeons found 2 types of tumors. Cystic tumors with massive bleeding invading the surrounding structures (the dura mater or jugular bulb) were more common in the sporadic cases. Fibrous tumors that infiltrate the bone and have moderate bleeding were more common in the cases associated with VHL disease.Two patients with small lesions were not operated on but were followed for 6 years without tumor growth. They died of metastasis from gastric and kidney cancer. Four recurrences occurred during the 14 years of follow-up. Four facial palsies and 8 cases of profound deafness were encountered postoperatively. Sporadic tumors are more aggressive than those associated with VHL disease. Complete surgical resection should be the goal of treatment. Preoperative angiography with embolization is recommended. In some cases, embolization may be impossible, and preoperative or postoperative radiotherapy should be discussed.

Meralgia paresthetica secondary to abdominal desmoid tumor

Meralgia paresthetica is a mononeuropathy of the lateral femoral cutaneous nerve that supplies sensation to the anterolateral portion of the thigh. Compression of this nerve can lead to paresthesias and neuropathic pain. It is commonly associated with pregnancy, obesity, trauma or diabetes mellitus. Here we present a case of meralgia paresthetica in a 26-year-old woman secondary to nerve compression by a desmoid tumor within the internal oblique muscle tissue. Desmoid tumors are fibrous neoplasms originating from musculoaponeurotic structures, and can be locally aggressive and recurrent.

Recurrent infantile digital fibromatosis

We present a case of an 8-year-old-boy with recurrent infantile digital fibromatosis (IDF) who presented with new fibrotic lesions. IDF is a benign fibrous growth of childhood. Typically affecting the fingers and toes of children, the condition is nonmalignant and has a high recurrent potential following surgical excision. Although B200 cases of IDF have been described to date, a generalized consensus does not seem to exist on an approach to treatment. The current treatment modalities for IDF are reviewed. Keywords: fibrous tumors, inclusion body fibromatosis, infantile digital fibromatosis, spindle cells, Reye tumor

A Case Report and Update of Giant Cell Fibroma

ABSTRACT A case report and update of giant cell fibroma The giant cell fibroma is a fibrous tumour with a distinctive clinicopathologic features which sets it apart from a conventional fibroma. It represents approximately 2% to 5% of all oral fibrous proliferations submitted for biopsy and is found predominantly in Caucasians and rarely in other races. Most often giant cell fibroma is mistaken for papilloma because of its papillary surface or fibroma because of its fibrous or elastic nature. Here, we report a case of giant cell fibroma, in the region of the incisive papilla, in a 30-year old autistic female. Key words: Giant cell fibroma, fibroma, histopathology, giant cells MUSBED 2014;4(1):58-62 Olgu Sunumu / Case Report INTRODUCTION The giant cell fibroma (GCF) is an interesting non-neoplastic lesion of the oral mucosa. It was first described by Weathers and Callihan in 1974. It was named for its characteristically large, stellate-shaped, mononuclear and multinucleated giant cells. Weathers and Callihan reviewed more than 2000 specimens at Emory University of which 108 specimens met the criteria for reclassification of GCF (1). Before Weathers’ and Callihans’ distinction of GCF, Eversole and Rovin compared and contrasted 279 fibrous hyperplastic gingival lesions, which falls into four categories: pyogenic granuloma, peripheral gingival fibroma, peripheral giant cell granuloma, and peripheral ossifying fibroma. Each has its own diagnostic histopathologic characteristics but exhibit overlap of clinical presentation (2,3). After distinguishing GCF among fibrous hyperplasias, Weathers and Campbell further elucidated the structure of the lesion when they studied them under light microscopy. They concluded again that dominant cells in the GCF were indeed unique, and that GCF merited its own classification (4,5). Giant cell fibroma is a fibrous oral benign asymptomatic pedunculated or sessile nodule less than 1 cm in size, more commonly seen in the mandibular gingiva and the lesion may persist for several years (6,7). The giant cell fibroma is a localized reactive proliferation of fibrous tissue, much like the irritation fibroma. It usually remains small and may have a broad base or be on a thick stalk. It is painless and often has lobules or nodules on its surface. The most characteristic histological feature is the presence of large spindle-shaped and stellate-shaped mononuclear cells and multinucleated cells (4). Very few case reports are seen regarding this tumour and controversy regarding the origin of this lesion continues (8). Here, we report a case of a 30-year old female who had

Paraspinal desmoid tumor in an 18 year old female

Desmoid tumors are benign neoplasms of fibroblasts characterized by local invasiveness. They often recur after surgical resection. They constitute about 3.5% of all fibrous tissue tumors and 0.03% of all neoplasms. Paraspinal desmoid tumors are rare and only few cases have been reported in the literature. We present an 18-year-old female presenting with a paraspinal mass. Patient had a history of trauma at the same site one year back. FNA (Fine needle aspiration) was done and a diagnosis of benign fibroblastic lesion was made which was later confirmed on histopathology. We present this case on account of its rarity and its importance in the differential diagnosis of post traumatic paraspinal masses.

An incidental retroperitoneal solitary fibrous tumor

Abstract Solitary fibrous tumors (SFTs), are a group of rare, spindle-cell derived fibrous neoplasms most commonly found in the thoracic (pleural) cavity (80%). However, it has been described that SFTs can affect a wide range of

Acquisition of Mouse Tumor Biopsies through Abdominal Laparotomy

The static analysis of tumor tissues at a single, terminal end point has been the mainstay of studies in mouse models of cancer. However, with the development of model systems that reproduce the intra- and intertumoral heterogeneity of human tumors, such studies are limited by the need for large numbers of animals to overcome increased intragroup variance. The shortcomings of a single-timepoint approach to molecular analysis are especially apparent in the context of therapeutic studies, in which the dynamic response to treatment is of particular consequence. To mitigate the effects of intertumoral heterogeneity, multiple tissue samples may be harvested from the same tumor at different time points through the use of surgical biopsies. For abdominal tumors, preprocedure imaging may be used to assess the suitability of tumors for biopsy. Sterile surgical techniques are used to access the abdominal cavity, and customized instruments facilitate the immobilization and retrieval of tissue samples from hard or fibrous tumors. Thermoregulation, hemostasis, and wound closure techniques are critical to successful surgical outcomes, whereas appropriate anesthetic, analgesic, and postoperative recovery regimens are important for maintenance of animal welfare. Using a mouse model of pancreatic ductal adenocarcinoma, we present a comprehensive protocol suitable for the routine acquisition of abdominal tumor biopsies.

Utility of a rotation–suction microdebrider for tumor removal in endoscopic endonasal skull base surgery

The microdebrider is a common tool used in endoscopic sinus surgery for removing polypoid and sinonasal tissue. It uses rotating blades and an integrated suction device for controlled removal of tissue under video–endoscopic visualization. To our knowledge, the application of the microdebrider for endoscopic removal of skull base tumors has not been reported. This study aimed to investigate the utility of the rotation–suction microdebrider as a tool for endoscopic endonasal removal of solid and fibrous skull base tumors. Thirty-two patients underwent endoscopic endonasal skull base surgery where the rotation–suction microdebrider was used as the primary tool for tumor removal and debulking. Pathologies included a variety of anterior skull base meningiomas, sinonasal skull base malignancies, juvenile nasopharyngeal angiofibromas, schwannomas, and other skull base lesions. Gross total and near total removal was achieved in 87.5% (28/32) of patients, and subtotal removal was performed in 12.5% (4/32) of patients. The microdebrider allowed efficient debulking and removal of solid and fibrous tumors, such as meningiomas, that were not responsive to standard ultrasonic aspiration. There were no complications of orbital or neurovascular injury, or thermal injury to the nostril. The rotation–suction microdebrider is a useful tool for endoscopic endonasal removal of skull base tumors. This is particularly useful for solid and fibrous tumors that are not responsive to standard ultrasonic aspiration. For intracranial tumors, it is critical to remain inside the tumor capsule during debulking so as to avoid injury to the surrounding neurovascular structures.

Rosai-Dorfraan disease in nasal cavity：two case reports and literature review

Objective To study the clinical manifestations, pathological features and differential diagnosis of extranodal Rosai-Dorfman disease （RDD） in nasal cavity. Methods The clinical, pathologic, immunohistochemical and special staining features of 2 cases of RDD were evaluated and related literatures were reviewed. Results Extranodal RDD in nasal cavity and various degrees of stromal fibrosis were seen association with mixed inflammatory cells （especially plasma cells）.The large polygonal histiocytes varied in number and were distributed in clusters or patches. Conclusion Extranodal RDD of nasal cavity is relatively rare and pathologic diagnosis may be difficult. It can easily mimic rhinoscleroma, hyperplasia of langerhans cells, granulomatous inflammation and fibrous tumors. Immunohistochemical and histochemical study is helpful in the differential diagnosis. Key words: Rosai-Dorfman disease; Nasal cavity; Histiocytosis; Immunohistochemistry; Differentialdiagnosis

Pediatric fibromatosis involving mandible: case reportand a five-year post-operative follow-up

Extra-abdominal fibromatosis is a benign fibrous neoplasm of locally aggressive behavior. Surgical excision with a wide margin is the treatment of choice. The aim of the present work is to report the case of fibromatosis in an 11-year-old melanodermic patient, who showed swelling in the area of the right mandible. Conservative surgery was performed based on the initial histopathological diagnosis of benign lesion suggestive of neural origin. The new anatomopathological examination of the surgical specimen was compatible with fibromatosis. The patient is still under periodic observation as part of the five-year surgical follow-up, showing no signs of recurrence.

Role of Ultrasound in the Diagnosis of Common Soft Tissue Lesions of the Limbs

The objective of this study was to investigate the role of ultrasound in the diagnosis of the soft tissue lesions of the limbs. Database of the soft tissue lesions of the limbs were reviewed retrospectively. All lesions had been confirmed by histopathology after surgical removal or biopsy. Ultrasound examination of the patients was performed by 4 sonologists with 9 to 22 years of experience, and the results were reviewed by 2 sonologists in panel. Categories and characteristics of some soft tissue lesions of the limbs were studied. Concordance rate was calculated, and reasons of misdiagnosis were analyzed. The patients consisted of 224 males and 173 females, and the age range was 6 months to 74 years (mean, 29.1 ± 27.3 years). The soft tissue lesions of the limbs included granuloma, inflammatory lesions, hematoma, hemangioma, neurofibroma, lipofibromatous hamartoma, fibrosarcoma, lymphangioma, liposarcoma, Baker cyst, epidermoid cyst, angiolipoma, fibrolipoma, malignant schwannoma, dermoid cyst, and intramuscular myoma. Fibrous tumor, lipoma, and hemangioma are of higher proportion. Baker cyst and neurofibroma were more often diagnosed correctly. Malignant lesions liposarcoma and malignant schwannoma were all misdiagnosed. A large number of misdiagnosed lesions were misdiagnosed as fibroma or lipoma. The overall concordance rate of sonographic diagnosis was 57.7%. Of the soft tissue lesions of the limbs, Baker cyst and neurofibroma are more often diagnosed correctly, and definitive diagnosis of other lesions is challenging. The overall concordance rate of sonographic diagnosis is not satisfactory.

Fronto - temporal approach in sellar and parasellar tumors

Abstract Introduction: The fronto-temporal approach represent the shortest distance to the ipsilateral optic nerve and carotid artery, and allow the direct verification of the optical nerves, the carotid arteries, the pituitary stalk, the tumor and its relationship with the suprasellar structures. Objective: The purpose of this study is to advocate an available cranial base technique for removing these tumors and to delineate the technique’s advantages that aid in achieving an improved extent of tumor resection and enhancing the patients’ overall outcome. Materials and methods: We present a retrospective study of a single surgeon experience on 355 consecutive cases with sellar and parasellar tumors admitted and operated by transcranial approaches in our department between January 2000 and December 2012. Results: Tumors in the sellar region represent 11, 8% of all tumors operated in our department. The most common type of tumor was pituitary adenomas, 165 of cases, followed by sellar and parasellar meningiomas, 128 of cases. Craniopharyngioma represent 12% of cases encountered in this region. All our cases underwent surgery by transcranial approach, unilateral frontotemporal in 252 of cases (71%). In pituitary adenomas total and near total resection was achieved in 100% of patients operated by transcranial approaches. For resection of craniopharyngiomas we have frequently chosen the extended fronto-temporal approach. The most common surgical related complications were: postoperative hematomas 9 of cases; wound infections, 6 of cases and CSF leakage, 12 of cases; transient visual alteration in 12 cases, transient third cranial nerve palsy in 6 of cases, transient motor deficit in 7 cases, hydrocephalus, 6 cases; transient diabetes insipidus in the large majority of pituitary adenomas and craniopharingiomas. Only 4 cases of pituitary adenomas and 6 of craniopharingiomas recurred after subtotal resection, requiring re-intervention. The mortality rate in our study was 2%. Conclusions: Our experience demonstrated that the management of the large suprasellar tumors via fronto-temporal approach remains the main route for these tumors with significant extrasellar extension, fibrous tumors, cases with unrelated pathology that might complicate a transsphenoidal approach and recurrent tumors after previous transsphenoidal surgery. In our opinion there is no need for larger osteotomies or extensive drilling of cranial base. “Instead of bone, remove the tumor; it's easier even for the patient”.

Cellular angiofibroma: two case reports of a rare vulvar tumor.

Cellular Angiofibroma of the vulva is a rare benign mesenchymal tumor of middle-aged women, reported first in 1997 [1]. Although it has predilection for the vulvar region, few extravulvular cellular Angiofibromas have also been reported. [2, 3]. It has a limited potential for local recurrence and is usually treated with complete local excision. The differential diagnosis includes Aggressive Angiomyxoma, Spindle cell lipoma, Mammary-type Myofibroblastoma, Angiomyofibroblastoma, Fibrous tumors, Perineuroma, and Leiomyoma [1, 4–6]. We are reporting here two cases of cellular Angiofibroma, both arising from the labia major, treated by complete local excision and with no recurrence so far in the follow-up.

Chéloïdes palmaire et plantaire chez un Noir africain

The keloid scar is a fibrous skin tumor, intradermal, and exuberant. It is commonly found on the glabrous skin. The keloid of the palms and soles are rare. Small series are reported in English literature. The authors report a case of large keloids located on both palms and soles, within a context of keloid disease, in a man of 37 years. On the left hand, the keloid scar caused a partial syndactyly IV-V. Large keloid tumors occupied the inner edge and, weight-bearing areas of both feet. These tumors rendered wearing of shoes impossible and interfered with walking. The treatment consisted of total excision of palmar and plantar keloid tumors. The residual defects were covered by a total skin graft taken from the suprapubic region. The results were satisfactory aesthetically, functionally and psychosocially.

A fibrotic nodule arising from the cerebellopontine angle

The authors present an extremely rare case of a fibrotic nodule arising from the cerebellopontine (CP) angle. A 57-year-old male had suffered from hearing disturbance and tinnitus for several years. Computed tomography revealed a high-density mass in the left CP angle with little enhancement after intravenous administration of contrast media. Magnetic resonance imaging (MRI) showed a very hypointense mass on T2-weighted imaging. T1-weighted MRI with gadolinium revealed very faint, delayed enhancement of the tumor. The patient underwent surgical resection of the tumor. Histopathologically the lesion comprised entirely fibrotic tissue consisting of thick collagenous fibers and sclerosing blood vessels with a few intervening viable cells with, partly, the immunophenotype of arachnoid cells. Intracranial fibrotic nodules are extremely rare. This tumor, however, had some radiological features similar to those of other, more common, tumors for example meningiomas or solitary fibrous tumors; it was, therefore, difficult to distinguish it from the others. It is believed that intracranial fibrotic nodules usually have benign, non-neoplastic characteristics, although their natural history is not yet fully understood. It is, therefore, necessary to be able to perform a differential diagnosis that will distinguish this rare condition from other intracranial fibrous neoplasms that occasionally have malignant features.

NY-ESO-1 expression in synovial sarcoma and other mesenchymal tumors: significance for NY-ESO-1-based targeted therapy and differential diagnosis

AbstractA promising targeted therapy against NY-ESO-1 (CTAG 1B) using genetically modified T-cells in synovial sarcomas was recently demonstrated in a clinical trial at the NCI. To investigate the role of NY-ESO-1 immunohistochemistry in patient selection and gain better insight into the incidence of NY-ESO-1 expression in synovial sarcomas and other mesenchymal tumors, we evaluated NY-ESO-1 expression by immunohistochemistry in 417 tumors. This collection of samples included: 50 SS18/SSX1/2 fusion positive synovial sarcomas, 155 gastrointestinal stromal tumors (GIST), 135 other spindle cell sarcomas as well as 77 other sarcomas (chondrosarcoma, osteosarcoma, dedifferentiated liposarcoma, alveolar soft part sarcoma, rhabdomyosarcoma, angiosarcoma, malignant mesothelioma, and Ewing’s sarcoma). We report that 76% of synovial sarcomas expressed NY-ESO-1 in a strong and diffuse pattern (2−3+, >50–70% of tumor cells). In contrast, only rare cases of other spindle cell mesenchymal tumor expressed NY-ESO-1 (GIST (2/155), malignant peripheral nerve sheath tumors (1/34), and dermatofibrosarcoma protuberans (2/20)). Individual cases of other sarcomas (angiosarcoma, malignant mesothelioma, chondrosarcoma, osteosarcoma, dedifferentiated liposarcoma, alveolar soft part sarcoma, and Ewing’s sarcoma) were positive for NY-ESO-1. However, no positive cases were identified amongst our cohort of leiomyosarcomas (0/24), hemangiopericytoma/solitary fibrous tumors (0/40), and cellular schwannomas (0/17). In summary, we find that NY-ESO-1 is strongly and diffusely expressed in a majority of synovial sarcomas, but only rarely in other mesenchymal lesions. Beyond its role in patient selection for targeted therapy, immunohistochemistry for NY-ESO-1 may be diagnostically useful for the distinction of synovial sarcoma from other spindle cell neoplasms.

Orbital Hemangiopericytoma/Solitary Fibrous Tumor in Childhood

A 12-year-old girl had a 6-year history of a large soft-tissue mass in her left orbit. The tumor biopsy was previously performed elsewhere when she was 7 years old, but no treatment was offered at that time. Later, the tumor was completely excised, and histologic examination revealed a mesenchymal neoplasia with typical hemangiopericytoma features. At 9 months of follow up, no evidence of local recurrence or metastasis was seen.

長期無再発のS状結腸直腸間膜原発solitary fibrous tumor（長径25cm）の1例

長期無再発のS状結腸直腸間膜原発solitary fibrous tumor（長径25cm）の1例

The MRI Findings of Collagenous Fibroma (Desmoplastic Fibroblastoma) Arising in the Supraspinatus Muscle: A Case Report

Collagenous fibroma (desmoplastic fibroblastoma) is a rare benign fibrous soft tissue tumor. This lesion commonly presents with painless, non-tender, mobile and slowly growing mass in subcutaneous tissue or skeletal muscle at various anatomic location. On magnetic resonance image (MRI), the mass shows area of low signal intensity on both T1and T2weighted pulse sequences. This low signal intensity presents areas of low cellularity in a background of abundant collagen. We describe the unique case of collagenous fibroma in the shoulder joint presenting with 4-years history of longstanding pain, which is unusual clinical manifestation of this benign soft tissue tumor without previous trauma history.

Giant cell collagenoma on the palm

ejd.2011.1521 Auteur(s) : Ji-Youn Park, You Chan Kim maychan@ajou.ac.kr Department of Dermatology, Ajou University School of Medicine, San 5 Woncheon-dong, Suwon 443-721, Republic of Korea Giant cell collagenoma (GCC) is a rare, benign, cutaneous, fibrous tumor that usually affects the face, trunk and upper limbs of young adults [1]. Since its first description, there have been only two additional case reports considered as GCC in the English literature [2, 3]. We report another case of GCC [...]