Fibrous Tumor Research Articles

Neurohypophyseal germinoma with abundant fibrous tissue

We report an unusual case of neurohypophyseal germinoma with abundant fibrous tissue and clival invasion that was initially misdiagnosed as lymphocytic hypophysitis. A 40-year-old woman presented with diabetes insipidus and panhypopituitarism after delivering her second son and which lasted for 4years. Magnetic resonance imaging showed the intrasellar mass extending to the suprasellar region with enlarged pituitary stalk. The mass was heterogeneously enhanced and invaded the clivus. Biopsy of the intrasellar mass was performed via the trans-sphenoidal route, and histological examination revealed marked fibrous tissue and infiltration of lymphocytes, with no evidence of tumor cells. Lymphocytic hypophysitis was the initial diagnosis, and corticosteroid therapy was begun. Despite intensive treatment, the lesion enlarged and clinical symptoms worsened 2weeks after surgery. Subtotal removal of the mass was performed, and a second histological examination revealed typical findings of the germinoma. Subsequently, the patient underwent chemoradiotherapy, and complete remission was achieved. Histological diagnosis is sometimes incorrect in fibrous tumors at the sellar region, and biopsy from several points is strongly recommended for this entity.

Hemangiopericytoma-Like Dermatofibroma With Mast Cells

We report an unusual case of hemangiopericytoma-like dermatofibroma in the right shoulder of an 82-year-old patient with a well-defined nodular growth located in the dermis. Microscopic study revealed a band of haphazardly arranged cells with a vascular component of gaping, simple, endothelial-lined vascular structures with intervening postcapillary venules and capillary-sized slit-like "staghorn" vascular channels filled with erythrocytes; abundant mast cells were also observed. The neoplasm cells were positive for CD68 and Factor XIIIA and negative for CD34. Few data have been published on the presence of abundant mast cells (tryptase and CD117 positive) in these neoplasm. The differential diagnosis of this entity should consider other spindle cell neoplasm, including hemangiopericytoma/solitary fibrous tumor, dermatofibrosarcoma protuberans, myopericytoma, angioleiomyoma, amelanotic melanoma, pecoma, and benign and malignant peripheral nerve tumors. We present an infrequent case of dermatofibroma with a vascular pattern resembling hemangiopericytoma and the presence of abundant mast cells, which may be responsible for this vascular component.

Sonography of Benign Palpable Masses of the Elbow

The purpose of this pictorial essay is to show the sonographic appearances of benign masses found in and around the elbow, including fat-containing tumors, fibrous tumors, vascular lesions, selected skin lesions, and other miscellaneous lesions. Sonographic features, including the use of dynamic and color Doppler imaging, can be helpful in characterizing these masses and in narrowing the differential diagnosis.

Abdominal wall desmoid tumor mimicking a subserosal uterine leiomyoma

Desmoid tumors are cytologically bland fibrous neoplasms originating from musculoaponeurotic structures throughout the body. The cause of desmoid tumors is uncertain, but may be related to trauma or hormonal factors, or may have a genetic association. These tumors can be found in some young women during pregnancy or just after giving birth. We report herein a case of desmoid tumor on the inner aspect of the abdominal wall that mimicked a large subserosal uterine leiomyoma. Initial clinical examination of the patient suggested a large abdominal wall tumor, while the imaging techniques including transabdominal ultrasound and magnetic resonance imaging suggested a large subserosal uterine leiomyoma as the initial diagnosis. This case emphasizes the importance of clinical examination during the diagnostic process.

A huge retroperitoneal solitary fibrous tumor

Solitary fibrous tumors (SFTs) are infrequent fibrous neoplasms that arise most commonly in the thoracic cavity. Although initially described as arising from the pleura, SFTs have been reported at a wide range of anatomic sites. Owing to their rarity, accurate diagnosis of SFTs remains a challenge for clinicians. A 38-year-old man presented complaining of difficulty in defecation and a progressive perineal mass. A computed tomography scan revealed a huge pelvic mass characteristic of central necrosis and calcification with a severely compressed adjacent rectum. Magnetic resonance imaging demonstrated a well-delineated mass with a maximal diameter of 15 cm. During surgery, an encapsulated tumor was excised via simultaneous abdominal and perineal approaches because of its huge size. SFT should be kept in mind for differential diagnosis of retroperitoneal tumors.

The role of chemotherapy in advanced hemangiopericyoma/solitary fibrous tumor.

10097 Background: Tumors comprising the histologic spectrum of hemangiopericytoma/solitary fibrous tumor (HPC/SFT) are rare soft tissue sarcomas with poor prognosis in the advanced setting. Little data exist regarding the response rate and progression-free survival in HPC/SFT patients treated with conventional chemotherapy. Methods: We retrospectively analyzed the clinical outcome of all patients with histopathologically confirmed HPC/SFT who were evaluated at our institution for treatment between 1994 and 2007. Descriptive statistics were performed from the data obtained. Best response to therapy was assessed using RECIST 1.1. Results: We identified 23 patients with measurable disease who received chemotherapy (M:F = 13:10; median age 50 (19-74). Nineteen patients had metastatic disease deemed surgically unresectable, while 4 patients had localized potentially resectable disease who received neoadjuvant chemotherapy. Fifteen patients received doxorubicin-based therapy, 4 patients received gemcitabine-based therapy, and 4 patients received paclitaxel therapy. Eighteen patients underwent treatment as first-line therapy and 5 patients as second-line therapy. The median number of cycles received was 4 (2-10). Reasons for stopping therapy included disease progression (n=11), toxicities (n=5), physician decision to give treatment break (n=3), surgical consolidation (n=3), and radiotherapy consolidation (n=1). Best response assessment using RECIST showed that 0/23 (0%) achieved complete response/partial response (CR/PR), 19/23 (83%) maintained stable disease (SD) ≥ 2 months , while 4/23 (17%) developed disease progression (PD). The median progression-free survival (PFS) was 4.6 months (1.3-15.7). Conclusions: Conventional chemotherapy has only modest activity in advanced HPC/SFT, as shown by the RECIST response rate of 0% and median PFS of 4.6 months in this retrospective series. Our findings can serve a reference for response and outcome in the assessment of novel agents in these histological subtypes.

HISTOPATHOLOGICAL CHARACTERISTICS AND IMMUNOCHEMICAL EXPRESSING IN EXTRAGASTROINTESTINAL STROMAL TUMORS

Background: Extragastrointestinal stromal tumors (EGISTs) are gastrointestinal stromal tumors(GISTs) that located in abdomen but outside the gastrointestinal tract. EGISTs is not common as GISTs with rate of 1-5%. Previously, EGISTs often diagnosed as gastrointestinal smooth muscle tumors, fibrous tumor or schwannomas. After the presence of the c-kit selective therapy administration(specified inhabition of CD117), the same as GIST, the EGIST are well responsed to this therapy, even when the tumors are at late stages such as invasion or metastasis. . Up to now immunohistochemistry has been indispensable for the diagnosis of EGIST in addition to the standard histopathology. Objectives: To classify the mesenchymal tumors in the abdomen outside the gastrointestinal tract. To study the Histopathological characteristics and Immunohistochemistrical expressing in EGIST. Materials and methods: discriptive research of 14 mesenchymal tumors in the abdomen outside the gastrointestianl tract. Using the inside and outside control for the immunohistochemistrical assessment. Results: 8/14 (57,1%) of these were GISTs, following the leiomyomas and leiomyosarcomas (21,3%). The others are less common including Schwannomas, fibromatosis and lipoma. The EGIST size: 10cm to 35cm, the average 19cm. The fusiform EGISTs accounting for 75%, the other types are less common, encompassing epithelioid and mixed tumors. Risk of Aggressive Behavior in EGIST: the high risk encompassing 100%. The positive proportion of the immunochemistrical markers: CD117 (100%), CD34 (62,5%), SMA (25%), S100 (12,5%). Desmin and CK negative in all cases.

Rare case of pelvic tumor – Aggressive fibromyxoma

The authors present a case of giant pelvic tumor in a 29-year-old previously healthy female. The tumor was an incidental finding during cesarean procedure after an unremarkably pregnancy. Both CT and MRI prior to surgery demonstrated a heterogeneous tumor measuring 30 cm × 16 cm × 5 cm occupying the entire pelvis and involving bilaterally both iliac and inguinal vessels. CT revealed little contrast enhancement and on MRI showed slight hypointensity signal on T1-weighted sequences and hyperintensity on T2-weighted sequences. Surgical excision was performed 2 weeks later. On follow-up the CT a few months later there was a recurrent tumor with the same location. She undergone angiography that showed no invasion of the superior and inferior mesenteric vessels and iliac. A pelvic MRI study was performed prior to the second surgery that showed a giant mass, from the iliac crest to the coccyx, pushing the uterus, bladder and rectum to the right, closed to gluteus muscles and along the left sacral roots without infiltrative aspects. The lesion demonstrated hypointensity on T1-weighted sequences and hyperintensity on T2-weighted images however failing to enhance with cystic-like features that are usually depicted in hemangiomas. A hypothesis of plexiform fibroma versus other fibrous tumors was then postulated. According to the imaging findings and the tumor recurrence the hypothesis of aggressive angiomyxoma considered most likely. The glass slide revision referred the differential diagnosis of cavernous hemangioma with retroperitoneal angiomyolipoma and aggressive angiomyxoma. She undergone surgery and the pathological the final diagnosis was aggressive angiomyxoma.

Intracranial hemangiopericytoma: Case study with cytogenetics and genome wide SNP-A analysis

The tumor entity of hemangiopericytoma is not universally recognized as a nosological entity by pathologists, and there is a trend toward reassigning it to other categories gradually. However, hemangiopericytomas occurring in the nervous system are included in the new WHO classification of brain tumors, and are distinguished from both meningioma and fibrous tumors. Since there are few genetic studies, we performed a comprehensive cytogenetic analysis of an infratentorial hemangiopericytoma in a 55-year-old female. It was originally classified as a grade II tumor but recurred as a grade III tumor with a proliferation index of 20%. Using trypsin-Giemsa staining (GTG-banding) and multicolor fluorescence in situ hybridization (M-FISH), we could confirm the loss of chromosomal material 10q, which has been previously described in hemangiopericytoma, and we identified de novo chromosomal aberrations on chromosome 8. Applying genome-wide high-density single nucleotide polymorphism array (SNP-A) analysis, we detected segments with loss or gain, as well as clonal deletions or regions suggestive of segmental uniparental disomy. These findings, together with the results of conventional histological and immunohistochemical characterization, provide additional evidence for the nosological separation of hemangiopericytoma in the central nervous system as a biologically different entity.

Extended endoscopic endonasal approach for selected pituitary adenomas: early experience

Whereas most pituitary adenomas are removable via the transsphenoidal approach, certain cases, such as dumbbell-shaped or suprasellar adenomas and recurrent and/or fibrous tumors, remain difficult to treat. The authors present their experience with the extended endoscopic endonasal approach to the suprasellar area in managing this subset of tumors, which are classically treated through a transcranial route. From June 1997 to December 2008, 615 patients underwent endoscopic endonasal transsphenoidal surgery for pituitary adenomas in the Department of Neurosurgery of the Università degli Studi di Napoli Federico II. Of this group, 20 patients with pituitary adenomas needed an extended endoscopic endonasal transtuberculum/transplanum approach for tumor removal. Two surgical corridors were used during the transsphenoidal approach: 1) the conventional endosellar extraarachnoidal corridor and 2) a suprasellar transarachnoidal corridor. The extent of resection was gross total in 12 (60%) of the 20 patients, near total in 4 (20%), subtotal in 3 (15%), and partial in 1 (5%). Postoperative CSF leakage occurred in 1 patient. One patient experienced worsening of temporal hemianopsia. The authors' initial results with the extended endoscopic approach to the suprasellar area for selected pituitary adenomas are promising and may justify a widening of the current classical indications for transsphenoidal surgery.

Calcifying Fibrous Pseudo Tumor arising from adrenal gland- a rare entity

Calcifying fibrous pseudo tumor is a rare, benign usually solitary fibrous lesion, commonly involving subcutaneous and deep tissues of the trunk, axilla, head and neck, inguinal and scrotal regions. We report this tumor presenting as an intra-thoracic mass, and found to be arising from the adrenal gland. Despite Computed Tomography (CT), the diagnosis evaded us till the biopsy report confirmed it. A wide excision was the key, and prevented residual or recurrent tumor in this patient. It is recommend to keep this entity in mind when dealing with a mass anywhere in the body, especially the thorax, for proper management.

Infantile fibromatosis: a case report and review of the literature

Desmoid tumours are benign fibrous neoplasms originating from musculoaponeurotic structures throughout the body. These tumours are rare in the mandible and the literature is limited to case reports and retrospective reviews and to date there is no agreed protocol for the management of these lesions in the paediatric mandible. The definition, diagnosis and management of juvenile fibromatosis still presents a challenge to the modern surgeon, radiologist and pathologist. We describe a case of paediatric mandibular infantile fibromatosis which presented a diagnostic dilemma, and review the currently available literature.

Minimally invasive neurosurgery for removal of pituitary adenomas by neuroendoscope aided with sellar floor reconstruction

To evaluate the effect of surgical technique and clinical curative effect for a unilateral endonasal-transsphenoidal approach in the removal of pituitary adenomas (PAs) by an endoscope-assisted technique with sellar floor reconstruction. Between April 2001 and March 2009, 426 consecutive patients underwent extended neuroendoscopic unilateral endonasal transsphenoidal surgery. The series consisted of 54 (12.7%) as I type of Pas and 105 (24.6%) II type, 181(42.5%) III type and 86 (20.2%) IV type according to Hardy's classification criteria of pituitary adenoma. Tumor removal, as assessed by intraoperative findings and postoperative magnetic resonance imaging or CT scans, revealed complete tumor removal in 370 cases (86.9%) and subtotal tumor removal in 32 cases (7.5%) were achieved. Partial removal was carried out in the remaining 24 cases (5.6%) with fibrous tumor. Three patients (0.7%) had postoperative death in this group. Twenty-four patients (5.6%) had a postoperative cerebrospinal fluid leak that required repair operations. Six patients (1.4%) experienced a sphenoid mucositis and meningitis and they were cured by medical therapy. The slightly soft texture of PAs with or without a limited suprasellar extension and without involvement of vascular structures, may be resected through such an extended neuroendoscopic transsphenoidal approach. Intraoperative angled endoscope is a useful adjunct for safe removals.

Characteristic endobronchial ultrasound image of hemangiopericytoma/solitary fibrous tumor

Hemangiopericytomatous pattern is characteristic of hemangiopericytoma/solitary fibrous tumor (HPC/SFT) and certain histological features might indicate a malignant potential, but the behavior of HPC/SFT is unpredictable. Endobronchial ultrasound (EBUS) is a useful diagnostic device in that the ultrasonographic image can be viewed and the EBUS-transbronchial needle aspiration can obtain a biopsied sample. We herein report a patient undergoing multiple surgical resections of recurrent HPC/SFT. A 74-year-old man had undergone right upper lobectomy for HPC/SFT 15 years ago. He received a partial resection of the left lung and a resection of the anterior mediastinal mass for its recurrences 13 years and six years ago, respectively. He had also undergone surgery for gastric carcinoma two years ago. He then presented with a tumor measuring 3 x 4 cm in the subcarinal area. Preoperative EBUS revealed a tumor with abundant thin-walled vessel-like structures, which was consistent with HPC/SFT. The tumor was completely resected and was finally diagnosed as low-grade malignant HPC/SFT.

(i) The epidemiology of primary skeletal malignancy

Abstract Primary skeletal malignancy is rare although in adulthood myeloma is not uncommon, with an estimated incidence of nine per million persons per year. It should not be confused with metastatic disease. The most common type of primary bony malignancy is plasmacytoma, after that chondrosarcoma, osteosarcoma and Ewing's sarcoma. The a priori chance of a patient to have a certain type of tumour depends on the patient's age. The most frequently seen under the age of 20 are osteosarcomas and Ewing's sarcomas. In adults, especially those over 40 years old, myeloma is the most common one, followed by chondrosarcomas and fibrous tumours. It is important for all treating patients with complaints of the musculoskeletal system to be aware of the possibility of primary bone cancer in order to prevent unnecessary delay and incorrect treatment.

Accuracy of dual-source computed tomography coronary angiography: evaluation with a standardised protocol for cardiac surgeons

Their comments allow us to report some additional information which was omitted due to space constraints. The mass was studied performing a 3-mm multidetector helical computed tomography (CT) scan, unfortunately without using a bolus tracking technique; an acquisition delay of 40 s, with a flow of 2.5 ml s 1 was applied. Themass appeared to be irregularly necrotic, and no significant arterial enhancement was evaluable (with the limits of the technique used). A critical revision of the acquired images was performed by an expert radiologist, and the hypothetic diagnosis of an encapsulated tumour such as a fibrous tumour or a leiomyosarcoma was addressed first. The possible theoretic utility in this clinical case of a magnetic resonance imaging (MRI), which was not performed, was posteriorly advocated according to the direct multiplanar imaging ofMRI, that, according to some authors [3], could help to delineate the diaphragm in certain anatomic areas that evade delineation on cross-sectional images, thus revealing its position in relation with the tumour. However, we completely agree with the colleagues that this examination is not a first-level tool in the diagnosis of intrathoracic masses, and its use should be reserved to selected cases, when diagnosis is not clear and further details could be important to achieve the correct diagnostic and therapeutic decision.

Inhibiting the VEGF–VEGFR pathway in angiosarcoma, epithelioid hemangioendothelioma, and hemangiopericytoma/solitary fibrous tumor

This review highlights the current body of knowledge regarding the role of the vascular endothelial growth factor (VEGF) and its receptor (VEGFR) in angiosarcoma, epithelioid hemangioendothelioma (EHE), and hemangiopericytoma/solitary fibrous tumor (HPC/SFT). Therapeutic agents that target this pathway are reviewed. Several phase II trials in advanced soft tissue sarcoma patients have investigated the efficacy of bevacizumab, an anti-VEGF antibody, as well as sunitinib, sorafenib, and pazopanib, VEGFR tyrosine kinase inhibitors (TKIs). Although response rates and progression-free survival periods were generally low, several angiosarcoma, EHE, and HPC/SFT patients demonstrated response or durable disease stabilization on these therapies. Biological mechanisms underlying the activity of these agents in angiosarcoma, EHE, and HPC/SFT are poorly understood. Some angiosarcoma tumors, however, harbor specific activating mutations in VEGFR2, which may be effectively targeted by VEGFR TKIs. Inhibition of the VEGF/VEGFR pathway may be a rational and effective therapy for certain patients with angiosarcoma, EHE, and HPC/SFT, but more studies are needed to confirm these findings and to identify which patients will benefit from these agents.

Cyst-like solid tumors of the musculoskeletal system: an analysis of ultrasound findings

When evaluating superficial soft tissue masses with ultrasonography, it is not always clear whether the mass is cystic or solid. We reviewed sonograms of soft tissue masses, which were interpreted as cystic but confirmed as solid tumors. We scanned a database of patients with superficial soft tissue masses from two different institutions. Four hundred and thirty lesions were evaluated with ultrasonography and then confirmed surgically. Twenty-three masses were identified, of which initial interpretation on gray scale included cystic tumor which pathology revealed to be solid tumors. All images were evaluated by two musculoskeletal radiologists, regarding internal echogenicity of lesions, presence of posterior enhancement, margins, size, and internal vascularity. Of 23 masses, there were 5 giant cell tumors of the tendon sheath, 4 schwannomas, 3 vascular leiomyomas, 2 benign fibrous histiocytomas, 2 dermatofibrosarcoma protuberans, 2 granular cell tumors, 1 dermatofibroma, 1 fibroma of the tendon sheath, 1 fibromatosis, 1 eccrine spiradenoma, and 1 granulation tissue. Masses were hypoechoic in 16 and anechoic in 7 cases. Posterior enhancement was present in 9, and 10 masses showed no internal vascularity on color Doppler imaging. Margins were smooth in 13, smooth with mild lobulation in 7, and lobulated in 3 cases. Mean size was 2.3 cm. On ultrasonography, solid soft tissue tumors may be confused with cystic masses. Such cases are not common, and fibrous tumors are the most frequent. Care should be given to these solid tumors that have a cystic appearance and even lack internal vascularity on color Doppler imaging to enhance diagnosis of cystic and solid masses.

Desmoplastisches Fibroblastom bei einer 22-jährigen Patientin im Bereich des Oberarms

Desmoplastic fibroblastoma ("collagenous fibroma") is a rare benign, slowly progressive fibrous soft tissue tumor that ranges 1-20cm in size. The tumor occurs in all ages, predominantly in the upper part of the body. Children are rarely affected. The tumor often infiltrates the subcutis; less often it invades the fascia and skeletal muscle or causes bone erosions. Histologically, the tumor is composed of spindle-shaped cells embedded in a densely collagenous stroma, sometimes forming a pseudo-capsule. Magnetic resonance imaging and computed tomography as well as ultrasonography are used to define the extensions of the tumor. Because it is clinically extremely difficult to determine if soft tissue tumors are benign or malignant, all should be excised with a margin of safety and examined histologically. We report a 22-year old woman with desmoplastic fibroblastoma located on the upper arm. Clinical and histopathological findings and treatment options are discussed.

An extremely rare case of a nasal turbinate lipoma

I would like to draw your attention to a very interesting case of a nasal turbinate lipoma that I encountered recently. This entity has never before been described in the literature. A 40-year-old woman with a known psychiatric disorder came for a routine MRI of the brain. Although there were no abnormal findings in the brain, I noticed a lobulated right nasal turbinate mass of uniform fat intensity (Figure 1a). CT showed a uniform fat-density lesion within the right inferior nasal turbinate, with no calcification or osseous components (Figure 1b). The features were suggestive of a nasal turbinate lipoma. Although histopathological correlation was desired, because of the asymptomatic nature of the lesion and the associated comorbid illness of the patient, no intervention was performed. However, the imaging features were themselves characteristic of a lipoma. Figure 1 (A) Sagittal T1 weighted image showing a lobulated hyperintense mass (white arrow) arising from the nasal turbinate (B) Axial CT showing a uniform fat-density lesion within the right inferior nasal turbinate (white arrow). Lipomas are one of the most common tumours in the head and neck region, however, they are very rarely encountered in the sinonasal tract, and most lipomas are large with osseous metaplasia (termed osteolipoma).1 If the fat density is not conspicuous on imaging, they can resemble an inverted papilloma or a fibrous tumour.1 As lipomas are benign and do not recur, they may undergo ischaemia and develop calcification, in which case they have to be distinguished from an osteolipoma, which is caused by osseous metaplasia rather than dystrophic changes.1 Our case revealed a uniform fat-density lesion arising from the right nasal turbinate, with no osseous components or calcification. This has not been reported in the literature to date.