Sural flap reconstruction of lateral malleolus following undifferentiated pleomorphic sarcoma resection with 1-year follow-up.

We report a case of a 54-year-old female who presents with a gradually expanding mass at the right lateral malleolus. The diagnosis of undifferentiated pleomorphic sarcoma was made after a histopathological examination of the mass following a wide tumor excision. The defected soft tissue area was reconstructed using a local flap, reverse sural artery flap. Following the surgical management, multiple radiotherapy sessions were completed. The patient's follow-up result showed no signs of local recurrence or metastasis, and the wound was well-healed with no complications other than paresthesia in a small area at the posterolateral aspect of the ankle under the lateral malleolus. This case represents a rare form of malignant neoplasm and emphasizes the effectiveness and safety of the reverse sural artery flap reconstruction technique, especially in places where microsurgery is unavailable or when the patient's status does not allow for prolonged anesthesia.

Imaging and Clinical Findings of Primary Malignant Fibrous Histiocytoma of the Urinary Bladder: A Case Report.

Primary malignant fibrous histiocytoma (MFH) is a malignant tumor of mesenchymal origin that rarely occurs in the urinary tract, particularly in the urinary bladder. Unlike urothelial carcinoma, which accounts for most bladder cancers, it occurs in the submucosal portion of the bladder wall and consists of the lamina propria, muscularis propria, and adventitia. It is presumed to originate from poorly differentiated pluripotent mesenchymal cells in which fibroblasts and histiocytes are partially differentiated. Radiologically, it is known as the "non-papillary tumor" and is commonly diagnosed as a large mass without necrosis, which shows invasion beyond the muscularis propia. Although the prognosis of this rare malignancy depends on pathological parameters, it generally has a poor prognosis with high local tumor recurrence. Here, we present a case of primary MFH in the urinary bladder with clinical symptoms of lower abdominal pain without gross hematuria that recurred rapidly and showed an aggressive disease course.

Clinical Presentations and Intense FDG-avidity in Pulmonary Angiomatoid Fibrous Histiocytoma with Potential Diagnostic Pitfalls: A Case Report and Literature Review.

INTRODUCTION: Angiomatoid fibrous histiocytoma (AFH) is a borderline tumor usually affecting the the children or young adults. 18F-Fluorodexoyglucose (FDG) positron emission tomography/computed tomography (PET/CT) investigations of pulmonary AFH are rare, and there are currently no reports of intense FDG uptake in AFH. CASE REPORT: We report an AFH that occurred in the lung of a 57-year-old woman. She presented with paroxysmal cough and occasional bloodshot sputum. 18FFDG PET/CT revealed a right parahilar nodule with intense FDG-avidity, middle lobe atelectasis, and several bilateral axillary lymph nodes with mild hypermetabolic activity. This patient underwent a right middle lobe lobectomy via video-assisted thoracoscopy. Histopathologically, the diagnosis was pulmonary AFH. She had an uneventful postoperative course, and the bilateral axillary lymph nodes regressed during postoperative follow-up. CONCLUSIONS: The clinical presentation and image findings of patients with primary pulmonary AFH may be potential diagnosis pitfalls. The diagnosis of lymph nodes or distant metastases should be approached with caution. To avoid misdiagnosis, biopsy with histological examination and immunohistochemichal staining should be performed as early as possible.

Recurrent benign fibrous histiocytoma of the bone benefits from denosumab followed by malignant transformation: a case report.

Benign fibrous histiocytoma of the bone (BFHB) is a rare mesenchymal tumor, representing less than 1% of all benign bone tumors. This controversial entity is characterized by a mixture of fibroblasts arranged in a storiform pattern, varying amounts of osteoclast-type giant cells and foamy macrophages. Curettage or simple resection is usually curative. However, it was reported that up to 11% of the patients suffer from relapse. Here, we report a case of malignant transformation of BFHB after long-lasting disease stabilization under denosumab therapy.

Pleomorphic Dermal Sarcoma.

Pleomorphic dermal sarcoma (PDS) is a rare cutaneous/subcutaneous neoplasm of purported mesenchymal differentiation that exists along a clinicopathologic spectrum with atypical fibroxanthoma (AFX). While PDS and AFX share histopathologic and immunohistochemical features, PDS exhibits deeper tissue invasion and has a higher rate of metastasis and local recurrence than AFX. Given its aggressive clinical course, early recognition and clinical management of PDS are essential for optimizing patient outcomes. This review aims to provide a brief overview of the clinicopathologic and molecular features, prognosis, and treatment of PDS.

Angiomatoid fibrous histiocytoma: primary intracranial lesion with thoracic spine metastasis and a malignant course. Illustrative case.

BACKGROUND: Angiomatoid fibrous histiocytoma (AFH) is an exceptionally rare soft tissue neoplasm. This tumor primarily presents as a benign soft tissue lesion in children with an average age of 14 years. The standard treatment regimen is wide local excision with interval follow-up. However, newer reports have demonstrated malignant potential with the possibility of intracranial metastasis. OBSERVATIONS: A 45-year-old male with no soft tissue primary tumor presented with a primary intracranial lesion and thoracic spine metastasis refractory to chemotherapy and radiation treatment. LESSONS: This report illustrates the potential for a highly malignant nature of metastatic AFH. In addition, the authors demonstrate an incidence of AFH in a middle-aged male without a primary soft tissue or skin lesion. This report highlights the importance of prompt treatment and excision for AFH, as there is still little understanding of successful options for systemic therapy.

An Unusual Case of an ALK-Negative Epithelioid Fibrous Histiocytoma in the External Auditory Canal.

This article represents the first reported case in the external auditory canal of epithelioid fibrous histiocytoma (EFH), a rare benign cutaneous epithelioid neoplasm. Immunostaining revealed uncommon negative staining for anaplastic lymphoma kinase (ALK) expression. This case and literature review outline the diagnostic strategy for this highly unusual neoplasm. Laryngoscope, 134:3371-3373, 2024.

Recurrent and metastatic cellular cutaneous fibrous histiocytoma: A case report and literature review.

Cutaneous fibrous histiocytoma (FH) is considered a benign dermal tumor. The cellular variant is rare and poorly documented. Besides presenting a high risk of local recurrence, it has a low but serious metastatic potential. We present a case of metastatic cellular FH and also review the literature on this tumor, given its unusual metastatic development. A 47-year-old male patient presented with a lesion in the anterior surface of the right thigh, which has been present since adolescence but had grown during last year. Anatomopathological evaluation revealed a cellular FH, and the lesion was completely removed. Six months later, tumor recurrence with multiple compartment muscle involvement and pulmonary metastasis were detected. Both lesions were completely resected and after 3 years of follow-up, the patient is asymptomatic and free of the disease. We conclude that FH should be carefully sampled to detect variants with high local recurrence rates or with some metastatic risk such as the cellular one. We recommend wide surgical resection and a close follow-up including chest x-rays or thorax computed tomography (CT) in all cellular FH cases with local recurrence.

168 Cases of aneurysmal dermatofibroma and 29 cases of hemosiderotic dermatofibroma: A clinicopathologic study.

OBJECTIVES: Aneurysmal dermatofibroma (ADF) and hemosiderotic dermatofibroma (HDF) are rare variants of dermatofibroma (DF) characterized by distinct histologic features. While HDF is traditionally considered a precursor to ADF, supporting evidence is limited, and the etiology remains unclear. A retrospective analysis of 2128 DF cases (2016-2019) was conducted to investigate the clinicopathologic characteristics of ADF, HDF, and other DFs. METHODS: Histopathologically diagnosed DF cases were examined for ADF and HDF. Univariate analyses were performed to compare clinicopathologic features. RESULTS: Among the cases, 168 (7.9%) were ADF and 29 (1.4%) were HDF. Aneurysmal dermatofibroma and HDF shared several common characteristics, including lower occurrence in females, larger size, and increased cellularity (all P < .0001). Notably, 29% of ADFs lacked hemosiderin deposition. Aneurysmal dermatofibroma primarily manifested on exposed areas (face and forearm, both P < .001). In contrast, 41% of HDFs occurred on the lower leg (P = .018), and all lower leg HDFs exhibited signs of venous stasis, distinguishing them from other HDFs (P < .0001). CONCLUSIONS: Our findings indicate a potential close relationship between ADF and HDF. Contrary to conventional beliefs, we also presented the possibility of ADF progressing into HDFs. Physical trauma may induce ADF, and HDFs may emerge from ADFs in conjunction with venous stasis in the lower extremities.

Epidermal inclusion cyst embedded in benign fibrous histiocytic lesion with prominent epithelioid morphology.

Benign fibrous histiocytoma also known as dermatofibroma is one of the common mesenchymal neoplasms. It commonly develops in young adult with female predominance and predilection for the extremities, particularly lower extremities. Implantation of epidermis in the dermis or subcutaneous tissues may lead to the formation of epidermal inclusion cyst, which is the most common type of epithelial cyst. Development of epidermal inclusion cyst within a benign fibrous histiocytoma is a rare occurrence. This is a unique case of two unrelated lesions.

Angiomatoid Fibrous Histiocytoma of the Chest Wall Protruding into the Thoracic Cavity Mimicking Metastasis in a Patient with Breast Cancer.

Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumor of intermediate malignancy and uncertain differentiation. To date, only four patients diagnosed with AFH located in the chest wall have been described. Herein, we describe a 44-year-old woman diagnosed with breast infiltrating lobular carcinoma. During the imaging study with positron emission tomography-computerized tomography scan, a 4 cm solid lesion located in the chest wall was identified. Fine-needle aspiration followed by surgical excision with intraoperative frozen section study was performed. The combined histomorphologic, immunohistochemical, and molecular findings confirmed the diagnosis of AFH. In this report, we describe, to the best of our knowledge, the first patient with synchronous AFH and breast cancer.

Case Report: Angiomatoid fibrous histiocytoma in the hand: a rare clinical presentation and diagnostic challenge.

Angiomatoid fibrous histiocytoma (AFH) is a rare tumor of mesenchymal origin occurring in young adults. Based on its clinical course, it is said to have an intermediate potential. We present a case of a 59-year-old woman with AFH in the hand that was difficult to diagnose. A benign soft tissue tumor was suspected on magnetic resonance imaging, and its size and open biopsy suggested nodular fasciitis or inflammatory myofibroblastic tumor. A diagnosis of AFH was eventually made based on the analysis of the resected specimens. The characteristic findings of histopathology and immunohistochemistry in this case were relatively poor, so fluorescence in situ hybridization contributed to making the correct diagnosis. Considering its prognosis, careful follow-up was decided upon without additional surgery. Our case is a challenging one because of its atypical presentation and inconclusive imaging and histopathological findings.

High-Frequency Ultrasound Imaging to Distinguish High-Risk and Low-Risk Dermatofibromas.

BACKGROUND: Dermatofibroma has various pathological classifications, some of which pose a risk of recurrence and metastasis. Distinguishing these high-risk dermatofibromas based on appearance alone can be challenging. Therefore, high-frequency ultrasound may provide additional internal information on these lesions, helping to identify high-risk and low-risk dermatofibroma early. METHODS: In this retrospective study, 50 lesions were analyzed to explore the correlation between clinical and high-frequency ultrasound features and dermatofibroma risk level. Based on their pathological features, the lesions were divided into high-risk (n = 17) and low-risk (n = 33) groups. Subsequently, an identification model based on significant high-frequency ultrasound features was developed. RESULTS: Significant differences were observed in the thickness, shape, internal echogenicity, stratum basal, and Doppler vascular patterns between the high-risk and low-risk groups. The median lesion thickness for the high-risk dermatofibroma group was 4.1 mm (IQR: 3.2-6.1 mm), while it was 3.1 mm (IQR: 1.3-4.2 mm) for the low-risk dermatofibroma group. In the high-risk dermatofibroma group, irregular morphology was predominant (70.6%, 12/17), the most common being dermis-to-subcutaneous soft tissue penetration (64.7%, 11/17), and heterogenous internal echogenicity was observed in the majority of cases (76.5%, 13/17). On the other hand, regular morphology was more prevalent in the low-risk dermatofibroma group (78.8%, 26/33), primarily limited to the dermis layer (78.8%, 26/33), with homogeneous internal echogenicity also being prevalent in the majority of cases (81.8%, 27/33). Regarding the Doppler vascularity pattern, 69.7% (23/33) of low-risk dermatofibromas had no blood flow, while 64.7% (11/17) of high-risk dermatofibromas had blood flow. CONCLUSION: High-frequency ultrasound is crucial in distinguishing high-risk and low-risk dermatofibromas, making it invaluable for clinical management.

Undifferentiated pleomorphic sarcoma of the spleen: a case report and literature review.

BACKGROUND: Undifferentiated pleomorphic sarcoma is an uncommon sarcoma and its presence in the spleen is even rarer, with only a handful of cases reported in English literature. It is typically only diagnosed following histological analysis. Its rarity also means that there is little consensus over ideal management. CASE PRESENTATION: This report presents a case of a 40-year-old Caucasian male who was found to have a splenic mass after presenting with non-specific abdominal pain and generalized malaise. Numerous imaging modalities were used which demonstrated a large partially solid and partially cystic lesion in spleen with no evidence of metastasis. As core biopsies were undiagnostic, he was planned for a diagnostic and therapeutic splenectomy. However, despite magnetic resonance imaging 11 days prior to his operation showed no evidence of liver metastasis, a massive splenic tumour with hepatic metastases was identified intraoperatively. An open splenectomy, distal pancreatectomy and liver metastasectomy was hence carried out. Histological analysis confirmed liver metastasis secondary to a splenic undifferentiated pleomorphic sarcoma. The patient recovered well and was discharged home. He presented again three weeks after his operation with lower back pain, abdominal pain and fever. Computed tomography demonstrated extensive recurrent disease burden in the peritoneum and liver. The patient passed away a month after surgery. CONCLUSION: Splenic undifferentiated pleomorphic sarcoma is a rare tumour which may pose a significant diagnostic challenge on both clinical and histopathological grounds. Following diagnosis and treatment, its aggressive nature often results in a poor prognosis. Current literature fails to delineate any superior management strategy to increase survival.

Difficulties in diagnosing angiomatoid fibrous histiocytoma of the head and neck region: A case report.

BACKGROUND: Angiomatoid fibrous histiocytoma (AFH) is a rare, slow-growing soft tissue tumor. It appears mostly on the limbs and trunk in children and young adults. The biology of AFH remains unclear because of the small number of reported cases. Diagnostic testing does not provide definitive results. It has two clinical forms, that differ in terms of gene expression and clinical prognosis. It is important to inform the laboratory which specific gene testing is necessary. Here, we describe a case of rare AFH in the submandibular region using a full genetic panel. CASE SUMMARY: A 13-year-old boy who had been misdiagnosed in the past 6 mo by his dentist visited our clinic because of a lesion in the submandibular area on the right side. The lesion was homogeneous and painless upon palpation. No skin discoloration was observed. Due to the non-specific radiological picture computed tomography (CT), magnetic resonance imaging (MRI), cone-beam CT (CBCT), and ultrasound-guided biopsy were performed. A venous malformation was suspected on the MRI. None of the tests provided a definitive diagnosis. Owing to the non-specific radiological findings, the patient qualified for surgical treatment. The surgical procedure included an excisional biopsy. The diagnostic testing was extended using gene rearrangements. The most distinctive gene translocation in diagnosing AFH is within the EWS RNA-binding protein 1 (EWSR1)-CREB-binding protein. However, in this case, the diagnosis was confirmed by a rearrangement within the EWSR1 gene testing. CONCLUSION: AFH in the submandibular location is rare, and surgical treatment with genetic evaluation defines AFH type that affects subsequent procedures.

Pulmonary Metastasising Aneurysmal Fibrous Histiocytoma: A Case Report, Literature Review and Proposal of Standardised Diagnostic Criteria.

An aneurysmal fibrous histiocytoma is a rare cutaneous soft-tissue tumour which accounts for approximately 0.06% of all dermatopathologies. Metastasis is exceedingly uncommon, to the point that there have only been eight reported cases in the scientific literature. We present the case of a 25-year-old male with a primary aneurysmal fibrous histiocytoma located in the nuchal region which exhibited rapid growth and abrupt ulceration over a short time span and showed signs of locoregional aggressive infiltration. A subsequent histopathological analysis confirmed the presence of diffuse solid and cystic pulmonary metastases. Further genetic sequencing verified LAMTOR1-PRKCD fusion. This case report seeks to review the existing literature on aneurysmal fibrous histiocytoma, discuss the challenges of differential diagnosis and propose standardised diagnostic criteria.

Histiocytic sarcoma of the palate: a case report.

Histiocytic sarcoma is a malignant proliferation of cells that exhibit morphological and immunophenotypic features of mature histiocytes. Owing to its rarity, its clinical features and standard treatment have not yet been established. This report describes a case of histiocytic sarcoma of the palate that developed in a 76-year-old man, the first report of an intraoral histiocytic sarcoma. An extended resection was performed; however, establishing the excision line was extremely difficult because assessing the tumour boundary on imaging was challenging and the tumour underwent dynamic gross morphological changes following biopsy. Complete resection is required to obtain a favourable prognosis for high-grade tumours with indistinct borders. In this case, an intraoperative rapid examination with frozen section analysis was performed along the planned excision line to completely resect the tumours exhibiting such behaviour. At 28 months postoperatively, the patient demonstrated no recurrence or metastasis; however, he is under careful monitoring.