[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.

Large vulvar fibroepithelial polyp and review of differentials.

Vulval fibroepithelial polyps (FEPs) are a rare type of vulval fibroblastic tumour commonly found in premenopausal women. It is important to obtain an accurate pathological diagnosis because, despite being benign, the condition shares some characteristics with malignant vulva lesions in its differential diagnosis. We present a case of young woman in her 20s with a giant FEP. After surgical excision, the patient did not manifest any signs of recurrence after 1-year follow-up. Our review focuses on the distinguishing characteristics of these rare neoplasms as we explore their differential diagnosis.

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.

[EWSR1-SMAD3 positive fibroblastic tumor: a clinicopathological analysis].

Objective: To investigate the clinicopathological features, immunophenotypes and molecular genetics of EWSR1-SMAD3 positive fibroblastic tumor (ESFT) with an emphasis on differential diagnosis. Methods: The clinicopathological data, immunohistochemical profiles and molecular profiles of 3 ESFT cases diagnosed at the Department of Pathology, Fudan University Shanghai Cancer Center from 2018 to 2021were analyzed. The related literature was also reviewed. Results: There were two males and one female. The patients were 24, 12 and 36 years old, respectively. All three tumors occurred in the subcutis of the foot with the disease duration of 6 months to 2 years. The tumors were presented with a slowly growing mass or nodule, accompanied with pain in 1 patient. The tumors ranged in size from 0.1 to 1.6 cm (mean, 1.0 cm). Microscopically, the tumors were located in the subcutaneous tissue with a nodular or plexiform growth pattern. They were composed of cellular fascicles of bland spindle cells with elongated nuclei and fine chromatin. One of the tumors infiltrated into adjacent adipose tissue. There was no nuclear atypia or mitotic activities. All three tumors showed prominent stromal hyalinization with zonal pattern present in one case. Focal punctate calcification was noted in two cases. The immunohistochemical studies showed that tumor cells were diffusely positive for ERG and negative for CD31 and CD34, with Ki-67 index less than 2%. Fluorescence in situ hybridization on the two tested cases identified EWSR1 gene rearrangement. The next generation sequencing analysis demonstrated EWSR1-SMAD3 fusion in all three cases. During the follow up, one patient developed local recurrence 24 months after the surgery. Conclusions: ESFT is a benign fibroblastic neoplasm and has a predilection for the foot, characterized by ERG immunoreactivity and EWSR1-SMAD3 fusion. Local recurrence might occur when incompletely excised. Familiarity with its clinicopathological features is helpful in distinguishing it from other spindle cell neoplasms that tend to occur at acral sites.

Case of recurrent superficial CD34-positive fibroblastic tumor.

Superficial CD34-positive fibroblastic tumor (SCPFT) is a recently described rare superficial mesenchymal tumor. SCPFT has a distinctive morphologic appearance, marked by significant nuclear pleomorphism, low mitotic rate, and diffuse CD34 positivity. SCPFT is underdiagnosed because of its rarity and misdiagnosis as sarcoma, with very few reported cases of local recurrence or metastasis. Recognition and awareness of SCPFT are essential for accurate diagnosis and appropriate clinical management. We describe here the case of a 37-year-old male who presented with a right calf mass diagnosed as SCPFT with subsequent local recurrence of the tumor.

EWSR1-SMAD3 positive fibroblastic tumor: a clinicopathological analysis / 中华病理学杂志

Objective: To investigate the clinicopathological features, immunophenotypes and molecular genetics of EWSR1-SMAD3 positive fibroblastic tumor (ESFT) with an emphasis on differential diagnosis. Methods: The clinicopathological data, immunohistochemical profiles and molecular profiles of 3 ESFT cases diagnosed at the Department of Pathology, Fudan University Shanghai Cancer Center from 2018 to 2021were analyzed. The related literature was also reviewed. Results: There were two males and one female. The patients were 24, 12 and 36 years old, respectively. All three tumors occurred in the subcutis of the foot with the disease duration of 6 months to 2 years. The tumors were presented with a slowly growing mass or nodule, accompanied with pain in 1 patient. The tumors ranged in size from 0.1 to 1.6 cm (mean, 1.0 cm). Microscopically, the tumors were located in the subcutaneous tissue with a nodular or plexiform growth pattern. They were composed of cellular fascicles of bland spindle cells with elongated nuclei and fine chromatin. One of the tumors infiltrated into adjacent adipose tissue. There was no nuclear atypia or mitotic activities. All three tumors showed prominent stromal hyalinization with zonal pattern present in one case. Focal punctate calcification was noted in two cases. The immunohistochemical studies showed that tumor cells were diffusely positive for ERG and negative for CD31 and CD34, with Ki-67 index less than 2%. Fluorescence in situ hybridization on the two tested cases identified EWSR1 gene rearrangement. The next generation sequencing analysis demonstrated EWSR1-SMAD3 fusion in all three cases. During the follow up, one patient developed local recurrence 24 months after the surgery. Conclusions: ESFT is a benign fibroblastic neoplasm and has a predilection for the foot, characterized by ERG immunoreactivity and EWSR1-SMAD3 fusion. Local recurrence might occur when incompletely excised. Familiarity with its clinicopathological features is helpful in distinguishing it from other spindle cell neoplasms that tend to occur at acral sites.

The role of endoscopic submucosal dissection in the management of gastric inflammatory fibroid polyps: a single-center experience

Background and aim: gastric inflammatory fibroid polypsconstitute only 0.1 % of all gastric polyps. They are usually amenable to resection by snare polypectomy. However,on rare occasions, these lesions may require resection byendoscopic submucosal dissection. This study aimed toevaluate the effectiveness and safety of endoscopic submucosal dissection in the management of gastric inflammatory fibroid polyps not amenable to resection with snarepolypectomy.Methods: a retrospective observational study of all consecutive patients who underwent endoscopic submucosaldissection for gastric inflammatory fibroid polyps betweenJanuary 2011 and December 2020 was performed.Results: there were nine cases of gastric inflammatoryfibroid polyps resected by endoscopic submucosal dissection. Most patients were female (7/9) with a mean ageof 62.2 years. All gastric inflammatory fibroid polyps weredescribed as solitary antral subepithelial lesions with amean diameter of 16.7 mm, which appeared well-circumscribed and homogeneous lesions located at muscularismucosa and submucosa without deeper invasion on endoscopic ultrasound. All lesions were successfully resected byen bloc and complete resection with free margins obtainedin 8/9 specimens. Adverse events were reported in 2/9 casesincluding one intra-procedural bleeding successfully controlled with hemostatic clips and one aspiration pneumonia that evolved favorably. Mean follow-up duration was 33.7 months and no delayed complications or cases ofrecurrence were reported.Conclusions: endoscopic submucosal dissection appearssafe and effective for the resection of gastric inflammatoryfibroid polyps that present as large subepithelial lesions,if performed by experienced endoscopists after adequatecharacterization by endoscopic ultrasound, with high ratesof technical success and low recurrence rates. (AU)

Superficial CD34-positive fibroblastic tumor (SCPFT): A review of pathological and clinical features.

Superficial CD34-positive fibroblastic tumor (SCPFT) is a recently described rare mesenchymal tumor of borderline malignancy. It generally involves superficial soft tissue, with a predilection to the lower extremities. Microscopically this tumor is characterized by a fascicular and storiform growth pattern, spindled to epithelioid cells, nuclear atypia with pleomorphism, and eosinophilic granular, and fibrillar to glassy cytoplasm. Strong diffuse immunoreactivity for CD34 is very characteristic of this entity. Due to under-recognition, this tumor is generally underreported. Additionally, cases of recurrence are rarely reported in the literature. We will comprehensively review the English language literature on all reported cases of SCPFT, with emphasis on recurrence.

The role of endoscopic submucosal dissection in the management of gastric inflammatory fibroid polyps: a single-center experience.

BACKGROUND AND AIM: gastric inflammatory fibroid polyps constitute only 0.1 % of all gastric polyps. They are usually amenable to resection by snare polypectomy. However, on rare occasions, these lesions may require resection by endoscopic submucosal dissection. This study aimed to evaluate the effectiveness and safety of endoscopic submucosal dissection in the management of gastric inflammatory fibroid polyps not amenable to resection with snare polypectomy. METHODS: a retrospective observational study of all consecutive patients who underwent endoscopic submucosal dissection for gastric inflammatory fibroid polyps between January 2011 and December 2020 was performed. RESULTS: there were nine cases of gastric inflammatory fibroid polyps resected by endoscopic submucosal dissection. Most patients were female (7/9) with a mean age of 62.2 years. All gastric inflammatory fibroid polyps were described as solitary antral subepithelial lesions with a mean diameter of 16.7 mm, which appeared well-circumscribed and homogeneous lesions located at muscularis mucosa and submucosa without deeper invasion on endoscopic ultrasound. All lesions were successfully resected by en bloc and complete resection with free margins obtained in 8/9 specimens. Adverse events were reported in 2/9 cases including one intra-procedural bleeding successfully controlled with hemostatic clips and one aspiration pneumonia that evolved favorably. Mean follow-up duration was 33.7 months and no delayed complications or cases of recurrence were reported. CONCLUSIONS: endoscopic submucosal dissection appears safe and effective for the resection of gastric inflammatory fibroid polyps that present as large subepithelial lesions, if performed by experienced endoscopists after adequate characterization by endoscopic ultrasound, with high rates of technical success and low recurrence rates.

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.

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.

Spindle cell variant of epithelioid cell histiocytoma (spindle cell histiocytoma) with ALK gene fusions: Cases series and review of the literature.

BACKGROUND: Epithelioid fibrous histiocytoma (EFH) is an uncommon dermal neoplasm expressing anaplastic lymphoma kinase (ALK) protein. Rarely a histopathological variant of this entity exhibits exclusively spindle cells. We report three cases of EFH that do not completely fulfill phenotypic criteria featuring spindle cell morphology and expressing ALK protein. We also analyze the fusion partner genes rearranged with ALK in these cases. METHODS: ALK expression and rearrangement status were evaluated by immunohistochemistry (IHC), fluorescence in situ hybridization (FISH), and next generation sequencing based gene fusion analysis. RESULTS: Three cases, all from females between 25 and 55 years old, have been biopsied from back, left arm, and thumb. All three cases showed tumor with exclusively spindle cell morphology without any epithelioid cells. The tumor cells exhibited strong ALK expression by IHC and FISH study confirmed ALK gene rearrangement in all three cases. DCTN1-ALK fusion was identified in two cases. CONCLUSION: EFH is not always purely epithelioid and its spindled cell variant, spindle cell histiocytoma, should be included in the differential diagnosis of superficial dermal spindled cell neoplasms. ALK immunostain is a useful diagnostic marker for this entity and further studies may be useful to investigate whether DCTN1-ALK fusion mutations are specific to EFH with spindled cell features.

Fluorescence microscopy for the evaluation of elastic tissue patterns within fibrous proliferations of the skin on hematoxylin-eosin-stained slides.

BACKGROUND: Diagnosis of fibrous tumors can be challenging and expensive due to the use of special stains. OBJECTIVE: Determine the usefulness of fluorescence microscopy in the evaluation of elastic tissue patterns on hematoxylin-eosin-stained slides. METHODS: In total, 228 slides representing different fibrous tumors were evaluated for their elastic tissue patterns by fluorescence microscopy, and sensitivity and specificity were determined for relevant comparisons. RESULTS: Fluorescence microscopy was found to be useful, especially for distinguishing dermatofibroma from dermatofibrosarcoma protuberans and dermatomyofibroma from other fibrous tumors. LIMITATIONS: In some cases, excessive background staining made patterns difficult to interpret. CONCLUSION: Evaluation of elastic tissue patterns by fluorescence microscopy in fibrous tumors is a cheap and efficient means to further delineate these often challenging tumors.

EWSR1-SMAD3 rearranged fibroblastic tumor: Case series and review.

We report the largest series to date (N = 6) of EWSR1-SMAD3 rearranged fibroblastic tumor. Initially described in 2018, the tumor features a marked female predominance (F:M, 5:1, mean age 44-years, median age 45.5 years; range 27-57), with most cases (5/6, 83%) arising in acral locations (4 on foot/toe, 1 on hand). One case presented on the lower extremity. The lesions presented as nodules and were composed of short, variably cellular, intersecting fascicles of uniform spindled cells in a collagenous to myxoid stroma. In four cases, the tumor abutted the epidermis without a grenz zone. In one case, there was an abrupt transition to a central, acellular hyalinized area. Two other cases had admixed smaller collagenous areas, reminiscent of collagen rosettes. One had a concentric arrangement of tumor cells around blood vessels. Mitotic activity was low (<1/10 HPFs). All were positive for ERG by immunohistochemistry and negative for CD34 (6/6). An EWSR1-SMAD3 fusion was identified in three cases tested by next-generation sequencing (3/3). Rearrangement of EWSR1 by fluorescence in situ hybridization was showed in 1/1 case. Our series reaffirms prior findings and expands the known histopathologic spectrum of this emerging entity.

Agreement Between Clinical-Radiographic and Histopathological Diagnoses in Maxillofacial Fibro-Osseous Lesions

ABSTRACT Objective: To compare the agreement of clinical and radiographic diagnosis with the histopathological diagnosis in fibro-osseous lesions of the jaws. Material and Methods: An analytical and exploratory study was made based on systematic collected data, carried out in the laboratory of surgical pathology of a public Dental School. There were evaluated cases of fibrous dysplasia (FD), cemento-osseous dysplasia (COD) and ossifyng fibroma (OF), diagnosed by clinical, radiographic (panoramic and periapical radiography), and histopathological analysis, in a period of 12 years (from March 2001 to June 2013). Descriptive and inferential statistics (Fisher's exact test) were obtained. Results: Ninety-six cases of FOLs were evaluated. The radiographic aspects of the FOLs studied did not differ significantly (p=0.09). Radiolucent lesions were the least frequent, corresponding to approximately 13.5% of radiographic findings. Mixed lesions and radiopaques were more present, how they were COD and FD, respectively. The more aggressive variation of OF (Juvenile Ossifying Fibroma - JOF) was less frequent among the pathologies evaluated. In approximately 61.46% of the cases clinical and radiographic diagnosis were confirmed by histopathological diagnosis of FOLs. The highest agreement and the highest disagreement were observed in COD cases (40.7% and 62.2%, respectively). Conclusion: FOLs of the maxillaries represent a group of lesions in which the establishment of the clinical and radiographic diagnosis supported by the histopathological confirmation is critical and challenging.

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.

Pseudolymphomatous Atypical Fibroxanthoma.

Atypical fibroxanthoma is a rare mesenchymal skin tumor of intermediate malignancy that typically occurs on sun-damaged skin of elderly patients. Histologically, it is composed of pleomorphic cells with hyperchromatic nuclei and abundant cytoplasm, commonly arranged in a spindle cell pattern. Different histologic variants have been described during the past years. We present a case of atypical fibroxanthoma containing a dense inflammatory infiltrate, which in conjunction with the existence of immunoblast-like and Reed-Sternberg-like neoplastic cells could be misinterpreted as a lymphoid neoplasm. Immunohistochemical studies revealed strong positivity of tumor cells for CD10 and negativity for cytokeratins, p63, p40, S100, SOX10, ERG, actin, desmin, B and T-cell markers, BCL6, CD15, and CD30. The inflammatory infiltrate contained a mixed reactive T- and B-cell population with negative T-cell receptor and immunoglobulin heavy rearrangements. We discuss the differential diagnosis of this entity in which clinical, immunohistochemical, and molecular features are essential to avoid the diagnosis of a lymphoproliferative disease.

Superficial CD34-Positive Fibroblastic Tumor.

Superficial CD34-positive fibroblastic tumor (SCPFT) is a recently described entity that, despite significant pleomorphism, carries a good prognosis. We briefly describe this tumor and its principal differential diagnoses. Recognition of SCPFTs, including the clinical context in which they arise, is important to avoid confusion with other pleomorphic soft tissue tumors, particularly neoplasms in the group of pleomorphic sarcomas, which are typically aggressive tumors that could lead to unnecessary overtreatment.

Primary cutaneous solitary fibrous tumor with entrapped eccrine components.

First described in the pleura, solitary fibrous tumor (SFT) was subsequently reported in a variety of organ systems. Compared to other anatomic sites, primary cutaneous and superficial SFTs are relatively rare. Although several histopathologic variants of cutaneous and superficial SFT have been described, a primary cutaneous SFT harboring glandular components has not hitherto been documented in the literature. The current case report describes a spindle cell neoplasm of the right finger in a female patient with characteristic morphologic and immunohistochemical features of a SFT. Unexpectedly, various glandular and ductal components were identified in the SFT. A few hyperplastic lobules of sweat glands demonstrating similar morphology as the intratumoral glandular components and composed of secretory coils displaying mucinous metaplasia and ducts were detected in the attached subcutaneous tissue, suggestive of an inductive or obstructive effect of the SFT on the eccrine units. The intratumoral glands and ducts were judged to represent entrapped benign eccrine components from the attached subcutaneous tissue. Since this variant of SFT with entrapped eccrine components could mimic many biphasic epithelial and stromal tumors, pathologists should be aware of this unusual variant of SFT to avoid potential erroneous diagnosis, particularly mistaken confusion with a biphasic synovial sarcoma.

Cutaneous pleomorphic fibromas arising in patients with germline TP53 mutations.

Pleomorphic fibromas are rare benign cutaneous neoplasms associated with deletion/loss of chromosomes 13q and 17p, where RB1 and TP53 are located, respectively. Herein, we report five cases of pleomorphic fibroma arising in patients with germline TP53 mutations, suggesting a potential link with Li-Fraumeni syndrome. All three patients were female and young (mean age 27) with a strong personal and/or family oncologic history and confirmed pathogenic germline TP53 mutations. In two patients, multiple pleomorphic fibromas were diagnosed. Clinically, the lesions arose at various cutaneous sites and were small (≤2 cm) and raised (4/5). Histopathologically, the tumors were paucicellular, composed of atypical spindled to stellate cells with hyperchromatic and variably pleomorphic nuclei. Mitotic activity was exceedingly low, although rare atypical mitotic figures were seen in one case. Immunohistochemically, the tumor cells were diffusely positive for p16 (3/3) and showed loss of Rb expression (5/5). All cases showed aberrant p53 expression (overexpression in 4, complete loss in 1). The tumors have followed a benign clinical course with no evidence of progression or recurrence. In conclusion, the development of multiple pleomorphic fibromas in a young patient may be a clue to an underlying genetic cancer syndrome involving TP53.