[Intestinal calcifying fibrous tumor: case report]. / Tumor fibroso calcificante intestinal: reporte de caso.

Calcifying fibrous tumor (CFT) is a rare benign lesion of mesenchymal origin that may present similar characteristics to other more common tumors. We present the case of a 36-year-old woman with a tumor in the proximal jejunum, initially suspected to be a gastrointestinal stromal tumor (GIST). Surgical resection was performed, revealing a well-demarcated nodule at the anti-mesenteric border with microscopic features typical of a calcifying fibrous tumor. The tumor cells were positive for CD34 and negative for other markers, differentiating it from other neoplasms. Calcifying fibrous tumors can be confused with more common tumors because of its appearance, but an accurate diagnosis supported by immunohistochemistry is essential. Complete surgical excision is usually curative.

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.

Superficial CD34 Positive Fibroblastic Tumour with Myxoid Stroma.

Superficial CD34 positive fibroblastic tumor is a rare low-grade neoplasm of the skin and subcutis with indolent behavior. This entity has been included in the current World Health Organisation (WHO) classification of soft tissue tumors. Pathological diagnosis can be challenging due to significant morphological overlap with other entities and the large spectrum of CD34 positive tumors. We report a case in a twenty-five male which showed characteristic diagnostic features, but in addition showed myxoid stroma. The presence of myxoid stroma has not been previously emphasized in this entity and broadens the histologic differential diagnosis significantly to include myxoid soft tissue tumors. A subset of these tumors harbor PRDM10-rearrangements, but a defining molecular feature has not yet been described, highlighting the need for further molecular characterization of this potentially genetically heterogenous tumor. Awareness of this entity among surgeons and pathologists is important to prevent misclassification as an aggressive sarcoma and avoid over-treatment.

Multiple calcifying fibrous tumor of the pleura: A case report.

Calcifying fibrous tumor of the pleura (CFTP) is a rare benign tumor of the thoracic cavity. Due to the low incidence of CFPT, it is prone to be misdiagnosed because intraoperative analysis of frozen section is a challenge for pathologists. At present, it is difficult to distinguish this tumor from other benign thoracic tumors based on radiographic features. Therefore, surgical resection is the best method for definite diagnosis and treatment.

Pedunculated atypical fibroxanthomas of the face.

Atypical fibroxanthomas are rare, superficial dermal tumors. Most cases are benign and only locally destructive with a low rate of metastasis. Lesions are most commonly found on sun-exposed sites of elderly light-skinned patients and present as asymptomatic nodules with irregular borders; ulcerations and friability are other key characteristics. Pedunculated lesions, however, are rarely described in the literature. We present two cases of atypical fibroxanthoma manifesting as exophytic, pedunculated lesions on the face: one in a 74-year-old man and the other in an 82-year-old woman. These tumors are very effectively treated by excision with Mohs micrographic surgery.

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.

A case of long-term unchanged calcifying fibrous tumor.

A calcifying fibrous tumor (CFT) is a rare benign tumor that may occur in any part of the body. We report the case of an asymptomatic 21-year-old woman with thoracic tumors. Chest computed tomography showed multiple masses in the left costophrenic angle. As we were unable to diagnose the tumor with a CT-guided needle biopsy, we performed a thoracoscopic biopsy. We found smooth multilobulated masses on the pleura and multiple small nodules around the main tumors. Partial resection of the tumor was performed by VATS. Histological examination revealed that the tumor had hypocellular fibrosclerotic tissue and distributed psammomatous calcifications with inflammatory infiltrates. Immunohistochemistry demonstrated that the spindle cells were partially positive for CD34 and CD99, but negative for anaplastic lymphoma kinase-1, smooth muscle actin, BCL-2, STAT6, and S-100 protein. The tumor was diagnosed as CFT. The patient's postoperative recovery was uneventful, and no progression of the lesions was observed during follow-up.

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.

Surgical outcomes and clinical courses of solitary fibrous tumors of pleura.

AIM: This study's aim is to define the clinicopathological and surgical outcomes, clinical courses, and results of long-term follow-up of cases that underwent surgical treatment for solitary fibrous tumors of the pleura (SFTp). SUBJECTS AND METHODS: Clinical and long-term follow-up records of 16 consecutive patients who had surgery for SFTp between 2006 and 2016 were reviewed in the retrospective chart review. There were nine males (56%) and seven (44%) females with an average age of 60.5 ± 17.1 (range 30-87 years). Etiological factors, diagnostic procedures, clinical and surgical outcomes, 5-year overall survival (OS), and disease-free survival (DFS) in these patients were researched. RESULTS: There was no remarkable common etiological factor. Nine of the cases were asymptomatic. Other symptoms were chest pain, dyspnea, cough and hypertrophic osteoarthropathy, respectively. Thoracotomy for the removal of pleural mass was carried out in 15 (94%) cases. Additional resection procedures included the chest wall resection in two cases and lobectomy in two. Video-assisted thoracoscopic surgery (VATS) resection was performed in one (6%) case. Complete surgical excision was performed in 74% of cases. Nineteen percent of cases were malignant SFTp (mSFTp). One of the mSFTp cases died in the 19th month after the diagnosis. The mean follow-up time was 50.6 ± 34.2 months (2--114 months). Mean survival of mSFTp patients was 40.6 ± 19.08 months (19--55 months) and that for benign SFTp (bSFTp) was 52.9 ± 37.05 months (2--114 months). Five-year OS--DFS were 93.5% and 74%, respectively. CONCLUSION: SFTp is an uncommon benign neoplasm but it can have malignant features. Even in the case of recurrence, the main treatment is total surgical excision. Oncologic treatments can be tried in unresectable and metastatic cases. VATS can be used in surgical total excision for small diameter and appropriate tumors. Understanding the nature of these tumors, immunohistochemical, and genetic studies may be a guide in future.

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.

An Atypical Fibroxanthoma and Intradermal Nevus Collision Tumor-Potential for Misdiagnosis.

Atypical fibroxanthomas (AFX) are rare cutaneous tumors, which typically present as a solitary ulcerated papule or nodule on sun-damaged skin. Despite malignant-appearing features on histology, AFX typically pursue a benign clinical course. In rare instances, AFX can form collision tumors with other lesions. However, to the best of our knowledge, AFX in collision with a nevus has never been previously reported. In this study, we describe such a lesion for its novelty and challenge in diagnosis, as this case was originally considered to be melanoma arising in a nevus. On histologic examination, there were 2 distinct populations of cells; one composed of markedly atypical and pleomorphic epithelioid and oval to spindled cells, consistent with AFX, and the other, a bland-appearing intradermal nevus with congenital features. The AFX population stained positive with smooth muscle actin, CD10, and CD68 and was negative for S100, SOX10, Melan-A, desmin, pancytokeratin, CK5/6, and p63. Deep to this was a second population of small, bland-appearing melanocytes in a broad, band-like distribution. This unusual collision tumor between AFX and an intradermal nevus highlights the important role immunohistochemistry plays in avoiding the misdiagnosis and potential overtreatment of benign or low-grade lesions, and in identifying potential mimickers.

Atypical Fibroxanthoma.

Atypical fibroxanthoma (AFX) is a dermal spindle-cell sarcoma that is considered a superficial and clinically benign presentation of pleomorphic dermal sarcoma, malignant fibrous histiocytoma, and undifferentiated pleomorphic sarcoma. AFX appears clinically as a discrete red or pink nodule or papule, most commonly on the head and neck region of sun-damaged elderly patients. Histologic findings on routine hematoxylin and eosin staining reveal spindle-shaped, large, and pleomorphic tumor cells throughout the dermis. Immunohistochemistry is not specific for AFX, and the diagnosis is generally one of exclusion. AFX is best treated by complete surgical excision, with Mohs micrographic surgery considered the treatment of choice. Metastasis rarely occurs, but there is a high rate of local recurrence, especially in patients who are immunosuppressed.

Calcifying fibrous tumor of the terminal ileum mesentery: Case report.

RATIONALE: Calcifying fibrous tumors ("CFT") are recognized as extremely rare mesenchymal tumors with benign biological behavior and low rates of recurrence are seen after removal. The first case of a CFT was reported in 1988 as a possibly inflammatory triggered pseudotumor in deep soft tissue of children. Histologically, the tumor is typically composed of dense hyalinized collagen with paucicellular infiltration of lymphocytes and fibroblasts as well as psammomatous or dystrophic calcifications. It can affect soft tissue in very different anatomical locations, also intrathoracic and intra-abdominal, mimicking various different diagnoses. The etiology is understood to be unclear. Asymptomatic CFTs can be found incidentally on medical images. PATIENT CONCERNS: We present the case of a calcifying tumorous lesion found incidentally in the mesentery of the terminal ileum of a 34-year-old male patient in February 2016 undergoing a computed tomography for a urinary tract infection. DIAGNOSIS: Histopathological and immunhistochemical examination after surgery revealed a CFT. INTERVENTIONS: Our patient underwent lower abdominal median laparotomy for tumorectomy. OUTCOMES: Two years after surgery the patient is free of a recurrence. LESSIONS: We add another case of intra-abdominal CFT to medical literature to provide more information about this very seldom tumor. While the etiology of CFT should be further investigated, diagnosis and therapy seem clarified. CFT should be kept in mind as a rare differential diagnosis of calcifying tumors also in the abdominal cavity. Immunohistological work-up is important for finding the diagnosis and may also help solving pathogenetical questions.

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.

Large-sized pedunculated and polypoidal angiomyofibroblastoma of the vulva: A case report and literature review.

Angiomyofibroblastoma (AMF) represents a rare, benign mesenchymal tumor with a predilection for the vulvovaginal region, which may be misdiagnosed as aggressive angiomyxoma (AAM). Herein, we report a case of a 20-year-old nulliparous Chinese woman with a unique pedunculated and polypoidal mass, which had been developing within the previous 6 months in the left labium majus, exhibiting the AAM clinical impression but diagnosed as AMF. The mass measured 18 × 10 × 6 cm, and contained diffuse ulcerated areas and purulent discharge. A complete excision of the mass was performed. There was no subsequent evidence of recurrence, according to a 13-month follow-up. As a rare benign vulvovaginal tumor, AMF can present on patients of an early reproductive age with rapidly growing, polypoidal pattern. The whole exon sequencing analysis revealed the genomic alterations, which may contribute to the occurrence of AMF.