Calcifying Fibrous Pseudotumor Research Articles

Sclerosing angiomatoid nodular transformation of spleen with calcifying fibrous pseudotumor: A rare case with unusual association

Sclerosing angiomatoid nodular transformation (SANT) of the spleen is a rare benign vascular lesion of the spleen that was first described in 2004. SANT is associated with other concurrent diseases, mostly malignancies. Calcifying fibrous pseudotumor (CFPT) is a tumor-like lesion usually arising from soft tissue or peritoneal sub-serosa. Both SANT and CFPT are considered to be variants of the inflammatory myofibroblastic tumor. We report the rare case of a 24-year-old female presenting with a left abdominal mass that was clinically diagnosed as an extraintestinal gastrointestinal stromal tumor (GIST). Histopathological examination revealed SANT of spleen and CFPT. We report this case due to its rarity of occurrence and unusual association of SANT with CFPT.

Calcifying fibrous pseudotumor in the pelvic cavity: A case report and review of the literature

Calcifying fibrous pseudotumors (CFPs) are rare benign lesions mostly occurring in the limbs, necks and gastrointestinal tracts of young adults. In the present study, we report the first known case of a pelvic CFP in China in a 67-year-old male patient. The solitary tumor had an extremely regular shape and was in the vicinity of the sigmoid colon. Surgical resection was performed and the tumor was histologically diagnosed as CFP. We reviewed 64 cases of CFPs and discussed the clinical features of this disease in China and other countries. We found that the CFPs were most common in patients aged 18-60 and often had no obvious symptoms. They were immunoreactive for vimentin and factor XIIIa, while negative for CD117 and S100. In China, CFPs were mostly identified in the stomach, possibly associated with Chinese eating. The pathogenesis of this disease remains unclear. More research is needed to confirm the present results.

Calcifying fibrous pseudotumor of the adrenal gland: A rare case report

Calcifying fibrous pseudotumor (CFP) is a rare benign soft tissue lesion. We herein present an extremely rare case of CFP of the adrenal gland. A 32-year-old male patient presented to our hospital with a tumor-like lesion in the area of the left adrenal gland detected by ultrasound during a routine check-up several days prior. Contrast-enhanced magnetic resonance imaging examination revealed a solitary, well-circumscribed mass, in close proximity to the medial arterial branch of the left adrenal gland. Histological examination revealed that the tumor consisted of sheets of varying amount of inflammatory cell infiltration; interstitial fibrosis and psammomatous calcifications were also observed. To the best of our knowledge, this is the third case of CFP occurring in the adrenal gland in the published literature. We herein report the findings of this case and review the two previously reported cases of CFP of the adrenal gland with valuable information.

Visual Diagnosis: A 31/2-year-old Boy With Sickle -Thalassemia and a Groin Mass

1. Aditi Bagchi, MD* 2. Ashraf Elnawawi, MD† 3. Swayam Sadanandan, MD‡ 1. *Resident (PGY-3), Pediatrics, The Brooklyn Hospital Center, Brooklyn, NY. 2. †Attending Physician, Department of Pathology, The Brooklyn Hospital Center, Brooklyn, NY. 3. ‡Associate Chairperson, Department of Pediatrics, Chief, Division of Pediatric Hematology and Oncology, The Brooklyn Hospital Center, Brooklyn, NY. * Abbreviations: CFP: : calcifying fibrous pseudotumor IMT: : inflammatory myofibroblastic tumor A 3½-year-old boy with a history of sickle β-thalassemia presents to the hematology/oncology clinic for evaluation of a mass in his left groin. The child’s mother had noted a small lump in his left inguinal region 2 years ago. At that time, the pediatrician recommended observing the lump and monitoring any progression. Subsequently, the family moved out of town and did not seek further medical attention until now. Two weeks ago, his mother thought the mass had grown in size and brought him to a nearby hospital for evaluation. He was prescribed a 5-day course of oral antibiotics for a presumed local infection. However, there was no significant change in the size of the mass; so his mother brought him to the hematology/oncology clinic. There is no history of groin pain or erythema, abdominal pain, fever, weight loss, or change in bowel or bladder habits. There is no history of trauma. The child was born at term after an uncomplicated pregnancy and delivery, but newborn screening indicated sickle cell disease. On further evaluation, his mother was found to have β-thalassemia trait and his father had sickle cell trait. The patient was diagnosed as having sickle β-thalassemia and placed on penicillin prophylaxis and folic acid supplementation. Since then, he has had four hospitalizations for febrile illnesses but no history of documented bacteremia. He has not had any vaso-occlusive crises. His motor milestones are appropriate for age, with the exception of speech delay. On physical examination, the child is well nourished …

Obstructive jaundice secondary to haemobilia from calcifying fibrous pseudotumor

Calcifying fibrous pseudotumor (CFT) is an uncommon lesion hat was first described by Fetsch et al. [1], and is charcterized histologically by hypocellular hyalinized collagenous issue with calcifications and patchy lymphocyte infiltration. t can develop at any site, but obstructive jaundice caused y abdomen CFT is extremely rare, and has never been eported. A 60-year-old man presented to our department with 1-month history of progressive jaundice. Laboratory data ere all in normal range except for elevated total biliruin (192 mol/L). Computed tomography (CT) showed multiple alcified nodules in the abdomen (Fig. 1a), obstruction of ilar bile duct and intrahepatic duct dilatation. A tentative iagnosis of hilar cholangiocarcinoma was made. Exploratory aparotomy was performed; during surgery, we found muliple round and oval nodules of diverse sizes disseminated hroughout the mesentery (Fig. 1b), though no hilar space ccupying lesions were visible. Unexpectedly massive blood lots were found in the common bile duct (CBD) after inciion for exploration. After removal of the blood clots, massive leomorphic cluster microcalcifications were seen under the choleochoscope in the intima of the CBD, which had destroyed he inner membrane of CBD and caused haemorrhage. Finally, holedochojejunostomy was performed and the largest nodules ere resected for pathological examination. The postoperative ourse was uneventful, and CFT was confirmed histologically Fig. 2).

Calcifying Fibrous Pseudotumor of the Breast

Calcifying fibrous pseudotumor (CFP) is classified as a benign fibrous lesion, and is a rare pathologic entity. Previous reports have described CFPs in the extremities, chest wall, pleura, scrotum, mediastinum, neck, and visceral peritoneum. We present the first reported case of a CFP in the breast. CFP should be considered in the differential diagnosis for patients presenting with coarse indeterminate calcifications of the breast.

Multiple Calcifying Fibrous Pseudotumor of the Pleura

Calcifying fibrous pseudotumor (CFP) of the pleura is a rare benign lesion. Eleven cases of CFP of the pleura have been reported, and multiple nodules were present in seven cases. We report one additional case of multiple CFP occurring in the right pleura. The patient was 35-year-old asymptomatic male who presented at our hospital with incidentally found multiple pleural nodules. Diagnosis was made by video-assisted thoracoscopic surgery and complete resection of all disseminated lesions was possible with followed thoracotomy. Although multiple lesions may prevent the complete resection and CFP of the pleura is considered as benign lesion, complete surgical resection of all lesions seems to be the best therapy for CFP of the pleura to reduce additional dissemination and local recurrence.

Oral Calcifying Fibrous Pseudotumor: Case Analysis and Review

Calcifying fibrous pseudotumor (CFT) is a rare distinct soft-tissue lesion characterized histologically by lymphoplasmacytic aggregates in a rich collagenized background with abundant psammomatous and dystrophic calcifications. Occurring most often in children and young adults, CFTs are clinically benign lesions that can form over a broad anatomic distribution, including in subcutaneous and deep soft tissues, as well as in serosal and visceral locations. The cause and mechanisms of pathogenesis of CFT are unknown. Simple excision with a margin of normal tissue is the treatment of choice. The risk for local recurrence is low. In this article, we describe a case of CFT in a 29-year-old woman with a 7-cm mass on the right upper gingiva and hard palate, discuss the differential diagnosis with other oral spindle cell lesions, such as, desmoid fibromatosis, nodular fasciitis, inflammatory myofibroblastic tumors, solitary fibrous tumor and also review the recent literature on this rare benign entity.

Calcifying Fibrous Pseudotumor of the Spermatic Cord

344 Calcifying fibrous pseudotumor (CFP) is an unusual benign lesion of the soft tissue. CFP is regarded as related to inflammatory pseudotumor and it may sometimes represent a late stage of inflammatory pseudotumor, in that a CFP appears to be arising within an inflammatory pseudotumor. 1 This entity occurs predominantly in young adult patients and has a good prognosis. 2 CFPs are found mostly in the extremities, neck, trunk, pleura, and mediastinum, but rarely in the spermatic cord. 3-5

Calcifying Fibrous Pseudotumor of the Neck: Diagnostic Challenges of a Rare Benign Lesion

Neck masses, frequently encountered by physicians, comprise a vast range of diagnoses, with malignancy being the greatest concern. Calcifying fibrous pseudotumor (CFP) is a rare lesion with unknown pathogenesis, characterized pathologically by a predominance of abundant hyalinized collagenous tissue with focal lymphoplasmacytic infiltrate and psammomatous or dystrophic calcifications. We present the case of a 29-year-old woman who presented with a 4-cm left neck mass, accompanied by constitutional symptoms of vague weakness and lethargy. After the lesion failed to respond to a course of antibiotic therapy, fine-needle aspiration was performed, the pathology of which was indeterminate. The concern was that the lesion was a lymphoproliferative disorder-further workup was performed. CT of the chest, abdomen, and pelvis revealed no evidence of adenopathy or neoplasms. Subsequently, an incisional biopsy was performed, suggesting a diagnosis of CFP. Magnetic resonance imaging with contrast, performed to delineate the anatomy, revealed the lesion in the left neck, deep to the left clavicle, that extended superiorly into the supraclavicular fossa. Complete surgical removal of the lesion was successfully performed, with immunophenotyping confirming the initial diagnosis of CFP. We present a case report of cervical CFP, discuss the approach to neck masses, and review the recent literature on this rare, benign entity.

Calcifying fibrous pseudotumor causing thoracic outlet syndrome

Calcifying fibrous pseudotumor (CFP) is a relatively rare benign mass lesion that generally involves the soft tissues. We report a case of CFP occurring in an extremely rare location. The patient is a 34-year-old woman presenting symptoms of numbness of her right upper extremity caused by a CFP in her thoracic outlet and we discuss its clinical significance with a review of the literature.

A Pseudotumorous Nodular Lesion of the Subcutis in a Beagle Dog Comparable to Calcifying Fibrous Pseudotumor in Human

A subcutaneous mass was found in a 15-month-old male beagle dog. It grew to 6 cm in diameter, and then reduced gradually to 4 cm. Histopathologically, the mass was well circumscribed but uncapsulated and comprised of hypocellular hyalinized fibrosclerotic tissue and a variable inflammatory infiltrate, which was composed chiefly of the lymphocyte. The characteristic findings of this nodular lesion were remarkably similar to those of calcifying fibrous pseudotumor (CFP) in human, but lacking in calcification in this case. To establish the disease entity for this kind of fibrous pseudotumor in dogs, additional case reports are required.

Calcifying fibrous pseudotumor of lung: A previously unreported entity

Calcifying fibrous pseudotumor (CFP) is one of the most unusual benign tumors of childhood and is located mostly in soft tissues, pleura, and peritoneum. The first case of pulmonary CFP in a 7-year-old boy is presented. The clinicopathologic features of this rare entity are discussed with special emphasis on histopathologic features and prognosis including a brief literature review. There are no presumptive clinical and laboratory findings, including tumor markers and imaging techniques, that distinguish CFP from other space-occupying lung lesions. The most important diagnostic aid is to bear this entity in mind when a child presents with an unexplained pulmonary mass. Total excision is mandatory for the appropriate diagnosis and treatment.

Calcifying fibrous 'pseudotumor': clinicopathologic study of 15 cases and analysis of its relationship to inflammatory myofibroblastic tumor.

Calcifying fibrous pseudotumor (CFP) has been postulated to be a "late" stage of inflammatory myofibroblastic tumor (IMT). We analyzed 15 cases (8 females and 7 males; aged 1 to 65 years). The anatomic distribution was wide, including 3 cases each in neck, mesentery/omentum, and GI tract and 2 cases each in mediastinum and paratesticular sites. Follow-up information was available in 10 patients (range 4 to 228 months). Local recurrence occurred in 3 patients and was repeated in 2. Tumor size ranged from 0.6 to 25 cm. Lesions were well-circumscribed hypocellular spindle cell proliferations with dense stromal collagen, a lymphoplasmacytic infiltrate, and stromal calcifications. Features of conventional IMT were not seen. Immunostaining showed CD34 positivity in most cases as well as rare cells positive for smooth muscle actin and desmin, and consistent negativity for ALK-1 and S-100 protein. These findings suggest that CFP is a distinctive benign mesenchymal neoplasm with a low risk for recurrence and, therefore, best labelled as "calcifying fibrous tumor." There is no convincing evidence to support an association between CFP and IMT.

Calcifying Fibrous Pseudotumor versus Inflammatory Myofibroblastic Tumor: A Histological and Immunohistochemical Comparison

Calcifying fibrous pseudotumor (CFP), a recently described lesion, is characterized by a predominantly lymphoplasmacytic infiltrate with abundant hyalinized collagen and psammomatous or dystrophic calcifications. The cause and pathogenesis are unclear, but it has been postulated that CFP may represent a sclerosing end stage of inflammatory myofibroblastic tumor (IMT). We compared the histological and immunohistochemical profiles of seven cases diagnosed as CFP and seven as IMT. Histologically, the CFP demonstrated varying degrees of calcifications in addition to fibroblastic proliferation admixed with inflammatory cells composed of lymphocytes, eosinophils, and mast cells. The IMTs rarely contain calcifications and had a myofibroblastic proliferation varying from hyalinized acellular collagen to florid fibroblastic proliferations simulating sarcoma. The inflammatory component was composed primarily of plasma cells and lym-phocytes, sometimes arranged as lymphoid aggregates with germinal centers. All CFP cases were diffusely positive for factor XIIIa and negative for smooth muscle actin, muscle-specific actin, and CD34. All IMTs demonstrated diffuse positivity for actin, variable positivity for CD34, and focal positivity for Factor XIIIa. This study demonstrates certain distinct histologic, immunohistochemical, and electron microscopic features between IMTs and CFPs.

Immunohistochemical analysis of anaplastic lymphoma kinase expression in deep soft tissue calcifying fibrous pseudotumor: evidence of a late sclerosing stage of inflammatory myofibroblastic tumor?

Calcifying fibrous pseudotumor (CFT) is a rare benign soft tissue lesion composed of dense hyalinized fibrous tissue containing bland spindle-shaped cells admixed with a lymphoplasmacytic infiltrate and foci of dystrophic and often psammomatous calcifications. It has been suggested that CFT represents a late sclerosing stage of inflammatory myofibroblastic tumor (IMT). Recently, clonal cytogenetic abnormalities involving the anaplastic lymphoma kinase (ALK) gene on chromosome 2p have been identified in IMT, particularly those arising in deep soft tissue sites. We evaluated seven cases of deep soft tissue CFT diagnosed at the Cleveland Clinic Foundation and the University of Florida with available paraffin-embedded blocks using a monoclonal antibody to ALK (Dako, Carpenteria, CA) and a modified avidin-biotin complex method. The cohort included six women and one man with a median age at diagnosis of 43 years (range, 26 to 67 years). Sites of CFT included mesentery (3), peritoneum (1), omentum (1), serosa of small bowel (1), and anterior mediastinum (1). Immunohistochemically, only one case showed focal staining for ALK. The remaining six cases were negative, with appropriate positive and negative control staining. In conclusion, unlike IMT, CFT in deep soft tissue locations rarely expresses ALK by immunohistochemistry, suggesting that CFT is a different clinicopathologic entity than IMT, as opposed to representing a "burned out" IMT. Ann Diagn Pathol 5:10-14, 2001.

Case Report: Calcifying Fibrous Pseudotumor: An Ultrastructural Study

To clarify the pathogenesis of calcification in calcifying fibrous pseudotumor (CFT), the lesion arising in the chest wall of a 16-month-old boy was ultrastructurally investigated. Fibroblasts were surrounded by large amounts of collagen fibrils. The dystrophic and psammomatous calcifications were observed as electron-dense amorphous masses and laminated bodies, respectively, within the cytoplasm of fibroblasts and in the collagenous stroma. The degeneration of cytoplasm seemed to be an initial event of intracytoplasmic calcification. Extracellular calcified substances often abutted to fibroblasts. Fibroblasts may play an important role in both intracellular and extracellular calcifications of CFT.