Fibroblastic and Myofibroblastic Soft-Tissue Tumors: Imaging Spectrum and Radiologic-Pathologic Correlation.

Fibroblastic and myofibroblastic tumors are a variable group of neoplasms ranging from benign to malignant. These lesions may affect patients of any age group but are more frequently encountered in the pediatric population. Patient clinical presentation depends on the location, growth pattern, adjacent soft-tissue involvement, and pathologic behavior of these neoplasms. In the 2020 update to the World Health Organization (WHO) classification system, these tumors are classified on the basis of their distinct biologic behavior, histomorphologic characteristics, and molecular profiles into four tumor categories: (a) benign (eg, fibrous hamartoma of infancy, nodular fasciitis, proliferative fasciitis, fibroma of the tendon sheath, calcifying aponeurotic fibroma); (b) intermediate, locally aggressive (eg, desmoid fibromatosis); (c) intermediate, rarely metastasizing (eg, dermatofibrosarcoma protuberans, myxoinflammatory fibroblastic sarcoma, low-grade myofibroblastic sarcoma, infantile fibrosarcoma); and (d) malignant (eg, sclerosing epithelioid fibrosarcomas; low-grade fibromyxoid sarcoma; myxofibrosarcoma; fibrosarcoma, not otherwise specified). Detection of various components of solid tumors at imaging can help in prediction of the presence of corresponding histopathologic variations, thus influencing diagnosis, prognosis, and treatment planning. For example, lesions with a greater myxoid matrix or necrotic components tend to show higher signal intensity on T2-weighted MR images, whereas lesions with hypercellularity and dense internal collagen content display low signal intensity. In addition, understanding the radiologic-pathologic correlation of soft-tissue tumors can help to increase the accuracy of percutaneous biopsy and allow unnecessary interventions to be avoided. ©RSNA, 2023 Quiz questions for this article are available in the supplemental material.

Calcifying fibrous tumor: a rare case in the foot.

Calcifying fibrous tumor is a rare fibroblastic tumor with distinctive histological presentation that shows benign characteristics. To our knowledge, there are no prior reports that have documented imaging findings of calcifying fibrous tumor in the distal lower extremity. We report the case of a 25-year-old man who presented with a mass in the medial aspect of the right foot that was first noted 4 years earlier. Medical attention was sought due to perceived increase in size as well as increasing pain in the right foot. The patient had no limitations in activity but reported worsening discomfort while walking. An anteroposterior radiograph obtained at first presentation demonstrated a large calcified soft mass in the medial aspect of the foot. Contrast-enhanced MRI showed a mildly enhancing 6.5 cm × 2.5 cm × 8.5 cm mass, hypointense on T1- and T2-weighted images, infiltrating the adjacent abductor hallucis and flexor digitorum brevis muscles. Histopathology demonstrated multiple irregular fragments of white-tan firm tissue with a gritty cut surface, positive for CD34 on immunohistochemistry and consistent with calcifying fibrous tumor. Although rare in the extremities, this diagnosis should be considered in patients with a calcifying soft tissue mass. Low signal intensity with low-grade enhancement on MRI as well as stable disease course could prompt a diagnosis of calcifying fibrous tumor even in previously unmanifested locations.

Gastric calcifying fibrous tumors: Computed tomography findings and clinical manifestations.

ABSTRACT: To retrospectively analyze the computed tomography (CT) findings and clinical manifestations of gastric calcifying fibrous tumor (CFTs).The features of 7 cases with pathologically proven gastric CFTs who had undergone CT were assessed, including tumor location, contour, growth, degree of enhancement, calcification and clinical data. In addition, the size and CT value of each lesion were measured. The mean values of these CT findings and clinical data were statistically analyzed only for continuous variables.Four patients were female and three were male (mean age: 33.3 years; range: 22 ∼ 47 years). Nonspecific clinical symptoms: abdominal pain and discomfort were observed in four cases and the CFTs were incidentally detected in the other three cases. Regarding tumor markers, lower ferritin levels were observed in three female patients. All of the gastric CFTs were solitary and mainly located inside the body; they were in round or oval shape and exhibited endophytic growth. Gastric CFTs are usually small sized and could contain confluent and coarse calcifications; cyst, necrosis, ulcer, bleeding and surrounding lymphadenopathy were not found in any of the cases. Unenhanced CT values of gastric CFTs were higher than those of same-transect soft tissue. Mild-to-moderate enhancement in the arterial phase and progressive enhancement in the portal venous phase were mainly noted.A gastric mass with a high unenhanced CT attenuation value, confluent and coarse calcifications and mild-to-moderate enhancement could prompt a diagnosis of gastric CFT. In addition, (1) being young- or middle-aged, (2) having relatively low ferritin levels, and (3) tumor located in the gastric body have critical reference value for diagnosis of gastric CFT.

Calcifying fibrous tumor of the gastrointestinal tract: A clinicopathologic review and update.

Calcifying fibrous tumor (CFT) is a rare mesenchymal lesion that has been documented throughout the gastrointestinal tract. Gastrointestinal CFTs may occur at virtually any age, with a predilection for adults and for females. They occur most commonly in the stomach and the small and large intestines. CFTs are most often found incidentally, cured by local resection, and have a low risk of recurrence. Histology shows three characteristic features: Spindle cell proliferations within a densely hyalinized stroma, scattered calcifications, and lymphoplasmacytic inflammation. CFTs are immunoreactive for CD34, vimentin and factor XIIIa, helping to distinguish them from other benign mesenchymal neoplasms. The differential diagnosis of CFTs includes sclerosing gastrointestinal stromal tumor, leiomyoma, schwannoma, solitary fibrous tumor, inflammatory myofibroblastic tumor, plexiform fibromyxoma, fibromatosis, sclerosing mesenteritis, and reactive nodular fibrous pseudotumor. The pathogenesis of CFTs remains unclear, but some have hypothesized that they may be linked to IgG4-related disease, inflammatory myofibroblastic lesions, hyaline vascular type Castleman disease, sclerosing angiomatoid nodular transformation of the spleen, or trauma.

Complete resection of a giant calcifying fibrous tumor of myocardial origin.

A calcifying fibrous tumor (CFT) is a rare, benign tumor that commonly develops in the internal viscera. It is histologically characterized by hyalinized collagenous tissue with calcifications and lymphoplasmacytic infiltrates. There have been few reports of CFTs occurring in the heart. and the therapeutic approach of such cases has not been well established; however, complete surgical resection appears to be the best treatment option for cardiac CFT, since this lesion can cause cardiac symptoms and recurrence has been recently noted. To our knowledge, this report describes the largest cardiac CFT for which complete surgical resection was successfully performed.

Liposclerosing Myxofibrous Tumor in a Patient with Prostate Cancer: A Case Report.

CASE: A 64-year-old man diagnosed with prostate cancer was incidentally found to have a lesion in his distal femur. Although initially concerning for metastatic prostate cancer, after biopsy by an orthopaedic oncology specialist, a diagnosis of liposclerosing myxofibrous tumor (LSMFT) was confirmed. The lesion was treated with curettage and demineralized bone matrix grafting with close follow-up. CONCLUSIONS: This case report illustrates that LSMFT is not confined to the proximal femur and highlights the differences in radiographic appearance between LSMFT and more common metastatic bone lesions.

A solitary fibrous tumor: an entity to consider in the diagnosis of liver masses.

We report the case of a female admitted for abdominal tenderness and palpable mass identifying in abdominal ultrasound a big hyperechogenic lesion with anechogenic areas inside with a nonspecific contrast behaviour pattern that after removing was diagnosed of hepatic solitary fibrous tumor. Solitary fibrous tumor is an infrequent mesenchymal neoplasm, mostly beningn, with infrequent appearance in liver parenchyma. Clinical diagnosis of this entity is difficult because neither the laboratory data nor the radiological characteristics are specific and they are not well defined either, so anatomopathological and immunohistochemical study are needed, usually in the whole tumor because of its size frequently requires surgical removement.

Ultrasonographic imaging of calcifying fibrous tumor of cervical esophagus: A case report.

RATIONALE: Calcifying fibrous tumor (CFT) is a rare benign mesenchymal tumor characterized by cytologically benign fibroblasts that produce abundant collagen with scattered lymphocytes, plasma cells, and psammomatous or dystrophic calcifications. PATIENT CONCERNS: A 37 years old women was initially diagnosed thyroid nodule in local hospital. She was referred to receive microwave ablation of the nodule in our hospital. The patient was in good health with occasional foreign body sensation in the neck. Routine laboratory examination was normal. DIAGNOSIS: The lesion was suspected benign and originating from the esophagus rather than the thyroid gland by conventional ultrasound and contrast-enhanced ultrasound in our hospital and was finally proved to be CFT by pathology. INTERVENTIONS: The lesion was surgically removed. OUTCOMES: The patient recovered well and was followed up for 2 years without recurrence. LESSONS: Although imaging diagnosis of CFT is difficult, the findings of clear border, coarse calcification on conventional ultrasound and peripheral hypoenhancement without central enhancement on contrast-enhanced ultrasound (CEUS), together with real-time evaluation of the relationship between the tumor and esophagus help to distinguish CFT of cervical esophagus from other lesions in the neck.

Recurrent pleural effusion in a young woman: calcifying fibrous tumour of the pleura.

We present the case of a 23-year-old woman with a long-standing history of recurrent left-sided pleural effusion unrelated to her menstrual cycle. At her last presentation, non-contrast-enhanced chest CT showed a pleural effusion and a lower left hemithorax mass, both large enough to cause complete collapse of the lower lobe and partial collapse of the upper lobe. Thoracoscopic surgery revealed a multilobulated mass originating from the dome of the diaphragm with pleural deposits. Histopathology diagnosed this as a calcifying fibrous tumour of the pleura, a rare benign tumour with excellent prognosis when completely excised.

Preoperative Assessment of Pathologic Subtypes of Meningioma and Solitary Fibrous Tumor/Hemangiopericytoma Using Dynamic Computed Tomography: A Clinical Research Study.

BACKGROUND: Solitary fibrous tumors (SFTs)/hemangiopericytomas (HPCs) are highly vascularized tumors well known for malignant, invasive, and highly vascular features. To date, several studies have reported the preoperative imaging findings of SFTs/HPCs. In this study, computed tomography (CT) tumor values acquired from dynamic CT scan were selected to determine the tumor pathology of highly vascular tumors, such as SFTs/HPCs. METHODS: We conducted a retrospective study on patients with pathologically diagnosed meningiomas and SFTs/HPCs who had undergone a dynamic contrast CT scan. We assessed and compared the CT values of these tumors according to the pathology. RESULTS: From a total of 34 patients, 30 patients with meningiomas and 4 patients with HPCs were included. The mean CT values of SFTs/HPCs and angiomatous meningioma were statistically significantly higher than those of the other meningioma subtypes (P = 0.003). We also performed receiver operating characteristic curve analyses to detect an appropriate cutoff point for the CT value to differentiate tumor pathology, and the calculated threshold was 161 Hounsfield units (HU) (sensitivity, 100%; specificity, 75%; area under the curve, 0.87; 95%, CI 0.75-0.99). CONCLUSIONS: This study showed that obtaining a CT value is useful in determining highly vascular tumor pathology preoperatively. When considering neurosurgical extra-axial tumor removal, and when the CT value of tumors is >161 HU, then highly vascular tumors such as SFTs/HPCs or angiomatous meningiomas are likely, and this should be considered prior to surgical intervention and for risk assessment.

Gastric Calcifying Fibrous Tumor Manifesting as a Subepithelial Tumor.

A gastric subepithelial tumor is a common finding during upper gastric endoscopy. The differential diagnosis of such lesions is broad, and sometimes a rare disease can be diagnosed. A calcifying fibrous tumor (CFT) is a rare, benign mesenchymal tumor that usually affects children and young adults. To our knowledge, a CFT of the tubular gastrointestinal tract is very rare with fewer than 30 cases of gastric CFTs reported in the literature.

Ruptured Infantile Myofibroma of the Head Presenting in a Neonate: Case Report and Review of the Literature.

BACKGROUND: Infantile myofibroma/myofibromatosis (IM/M) is a myofibroblastic proliferative disorder often seen in infants and children. IM/M can result in congenital tumors of the head and neck and may occasionally present to the neurosurgeon. CASE DESCRIPTION: We report a case of a solitary ruptured myofibroma of the head in a newborn patient. The lesion was initially suggestive of encephalocele. We describe the presentation and management of this patient, including relevant imaging, histopathologic evaluation, and surgical technique. We subsequently review the literature of IM/M of the head and neck, highlighting the 3 forms of the condition, each requiring a distinct management strategy. CONCLUSIONS: Although this tumor rarely presents to the neurosurgeon, it may do so in the process of ruling out other more dangerous conditions. It is therefore important to consider this diagnosis in masses that occur in the head and neck of newborns.

Submucosal calcifying fibrous tumor of the stomach: A case report.

The calcifying fibrous tumor is a rare benign fibrous tumor which occurs in subcutaneous or deep soft tissues in children and young adults, but also is frequently seen in pleural and intraabdominal locations in older people. Gastric involvement has been only sporadically reported in the literature. We present here our experience with this unusual lesion discovered in a 68-year-old woman. Clinically, the tumor was described as a pendulating, submucosally located mass, in the body of the stomach on a lesser curvature. The calcifying fibrous tumor is a histologically distinct lesion composed of dense hyalinized collagen fibers, inconspicuous scattered fibroblasts, a varying amount of psammoma bodies or dystrophic calcifications and foci of lymphoplasmacytic infiltration. In this report we will focus on a brief review and differential diagnosis of this tumor and other more common or not widely known gastric spindle cell lesions.

Superficial CD34-positive fibroblastic tumor: Cytologic features, tissue correlation, ancillary studies, and differential diagnosis of a recently described soft tissue neoplasm.

Superficial CD34-positive fibroblastic tumor is a low-grade mesenchymal neoplasm of superficial soft tissues characterized by fascicles of spindle to epithelioid cells displaying nuclear pleomorphism and strong diffuse CD34 immunoreactivity. The intraoperative imprint cytology preparations (ICP) of a superficial CD34-positive fibroblastic tumor from a 50-year-old female are described. To the best of our knowledge, there is no report of the cytologic findings of superficial CD34-positive fibroblastic tumor in the English medical literature. The ICP, differential diagnosis, tissue correlation, and ancillary studies of this fascinating entity are discussed. Diagn. Cytopathol. 2016;44:926-930. © 2016 Wiley Periodicals, Inc.

Pediatric Fibroblastic and Myofibroblastic Tumors: A Pictorial Review.

Pediatric fibroblastic and myofibroblastic tumors are a relatively common group of soft-tissue proliferations that are associated with a wide spectrum of clinical behavior. These tumors have been divided into the following categories on the basis of their biologic behavior: benign (eg, myositis ossificans, myofibroma, fibromatosis colli), intermediate-locally aggressive (eg, lipofibromatosis, desmoid fibroma), intermediate-rarely metastasizing (eg, inflammatory myofibroblastic tumors, infantile fibrosarcoma, low-grade myofibroblastic sarcoma), and malignant (eg, fibromyxoid sarcoma, adult fibrosarcoma). Imaging has a key role in the evaluation of lesion origin, extent, and involvement with adjacent structures, and in the treatment management and postresection surveillance of these tumors. The imaging findings of these tumors are often nonspecific. However, certain imaging features, such as low or intermediate signal intensity on T2-weighted magnetic resonance images and extension along fascial planes, support the diagnosis of a fibroblastic or myofibroblastic tumor. In addition, certain tumors have characteristic imaging findings (eg, multiple subcutaneous or intramuscular lesions in infantile myofibromatosis, plaquelike growth pattern of Gardner fibroma, presence of adipose tissue in lipofibromatosis) or characteristic clinical manifestations (eg, great toe malformations in fibrodysplasia ossificans fibroma, neonatal torticollis in fibromatosis colli) that suggest the correct diagnosis. Knowledge of the syndrome associations of some of these tumors-for example, the association between familial adenomatous polyposis syndrome and both Gardner fibroma and desmoid fibromatosis, and that between nevoid basal cell carcinoma syndrome and cardiac fibroma-further facilitate a diagnosis. The recognition of key imaging findings can help guide treatment management and help avoid unnecessary intervention in cases of benign lesions such as myositis ossificans and fibromatosis colli. In this article, we describe the various types of fibroblastic and myofibroblastic tumors in children and the characteristic clinical manifestations, imaging features, and growth patterns of these neoplasms-all of which aid in the appropriate radiologic assessment and management of these lesions. (©)RSNA, 2016.

Unusual Presentation of Elastofibroma Dorsi on 18F-FDG-PET/CT: A Case Report.

A 70-year-old male patient underwent an Fluorodeoxyglucose-positron emission tomography-computed tomography for staging of a left parahilar lung neoplasm found during work-up for fatigue and asthenia. The scan demonstrated a hypermetabolic lung tumor, a hypermetabolic pleural effusion and 4 hypermetabolic bilateral soft tissue lesions of the chest wall corresponding to 4 elastofibroma dorsi. Initially, the oncologic disease was classified as stage IV because of the hypermetabolic pleural effusion. A transbronchial biopsy showed squamous cell carcinoma and the cytology of the pleural effusion revealed no malignant cells. As the other 4 hypermetabolic thoracic wall lesions were correctly diagnosed as benign despite their unusual presentation, the patient underwent surgery by left pneumonectomy and mediastinal lymphadenectomy. The lymph node involvement required adjuvant chemotherapy. Diagnostic confidence of the benignity of the hypermetabolic chest wall lesions allowed a more aggressive treatment with a better outcome after a malignant pleural effusion was excluded.

Hypervascular mediastinal masses: action points for radiologists.

Hypervascular mediastinal masses are a distinct group of rare diseases that include a subset of benign and malignant entities. Characteristic features and known association with syndromes and genetic mutations assist in achieving a diagnosis. Imaging allows an understanding of the vascularity of the lesion and should alert the radiologist and clinician to potential hemorrhagic complications and avoid percutaneous CT guided biopsies. In such cases, pre-procedure embolization and surgical biopsy maybe considered for better control of post procedure hemorrhage. The purpose of this article is to describe and illustrate the clinical features and radiologic spectrum of hypervascular mediastinal masses, and discuss the associated clinical and genetic syndromes. We will present an imaging algorithm to determine further evaluation and subsequently guide treatment.