Pediatric Plexiform Fibromyxoma: A Case Report.

The plexiform fibromyxoma is a rare mesenchymal tumor in adults that generally originates in the antrum of stomach, being its occurrence in pediatric patients exceptional. It was classified as a distinct entity by World Health Organization in 2010. No recurrences and metastases have been documented in many of the reported patients to date, being the surgical treatment curative. We report the case of a 3-month-old infant who presented to the emergency department with an episode of intestinal subocclusion requiring an emergent surgery. During the surgical intervention, a mass was identified in the jejunum, causing partial occlusion of its lumen. The surgical pathology report revealed an infiltrative tumor composed of spindle-shaped cells disposed in a stroma with a plexiform pattern alternating myxoid areas. These findings and the immunohistochemical characteristics of the neoplastic cells led to classify the tumor as a plexiform fibromyxoma. A description of the immunophenotype of this tumor is made and differential diagnosis with other gastrointestinal tumors is also discussed.

Pediatric fibromyxoid brachial plexus tumor with YWHAZ::PLAG1 gene fusion: a case report.

Pediatric fibromyxoid soft tissue tumors may be associated with gene fusions such as YHWAZ::PLAG1, with only three reported cases in the literature. We present the fourth case, a 13-year-old male with a pediatric fibromyxoid brachial plexus tumor with YWHAZ::PLAG1 gene fusion. This is also the first case to be reported in an adolescent, in the brachial plexus, and in the Philippines. The patient presented with a 10-year history of a slowly growing left supraclavicular mass and a 1-year history of intermittent dysesthesia in the left upper extremity. Neurologic examination was unremarkable. Imaging revealed a large left supraclavicular lesion with intrathoracic extension. Surgical excision was performed, and histopathology revealed a fibromyxoid tumor with YWHAZ::PLAG1 gene fusion. Although previous examples of this gene fusion pointed toward lipoblastoma as their primary pathology, our tumor does not completely fulfill the current diagnostic criteria for a lipoblastoma and may represent an intermediate form of the disease. Our case is unique not only because it is the first reported adolescent patient harboring such a lesion but also because of the relatively lengthy natural history exhibited by the tumor prior to its resection. This provided us with valuable information about its behavior, which suggests a more indolent growth pattern. This case also highlights the clinical importance of molecular testing of tumors, where recognition of disease entities can assist clinicians in deciding and advocating for the proper management.

Ovarian fibromatosis associated with large pedunculated fibroma in a 30-year-old woman: A rare coincidence or variant?

A 30-year-old nulligravida was referred under suspicion of large subserosal myoma. T2-weighted magnetic resonance imaging revealed multilobulated solid mass in the left lower abdomen measuring 16 cm in longitudinal diameter. The ovarian surface was covered with a marked T2-hypointense thick rim called "black garland sign," forming multiple nodular masses ranging from 1 to 5 cm in diameter in some portions of the bilateral ovaries. By laparoscopic-assisted minilaparotomy, the stalk of pedunculated mass originating from the left ovarian hilum was excised, followed by carrying out of the body after in-bag morcellation using a surgical scalpel. Right ovarian exophytic nodular masses larger than 1 cm were excised using monopolar electrode needle. Pathological examination of excised right and left masses showed fibroblast-like spindle cell proliferation with collagenous stroma; however, differences between right and left masses cannot be distinguished on a histological level. Postoperative diagnosis was ovarian fibromatosis coexisting with large pedunculated fibroma.

Rare cardiac tumour-nodular fasciitis.

Left ventricular tumour is a rare condition in children. The causes include vegetations, thrombus, and fibroma. 2-year-old asymptomatic female presented with an innocent heart murmur at 6 months of age. Subsequent follow-ups at 18 months of age showed left ventricular mass. Surgical pathology revealed "nodular fasciitis." This type of tumour has never been described in the heart before.

[Aortic Valve Papillary Fibroelastoma Stated for Preoperative Twelve Years without Any Symptom: Report of a Case].

We experienced a case of papillary fibroelastoma of aortic valve, which had been located for preoperative 12 years without any symptom. We could assess tumor growth rate of 0.17 mm/year. Because of recent reports of recurrence, close follow-up should be continued.

[A Case of Fibromatosis-Like Tumor Which Was Difficult to Differentiate from Local Recurrence of Ascending Colon Cancer].

A 60s female, who had undergone single-incision laparoscopic ileocecal resection for ascending colon cancer with pathological diagnosis of T3N1bM0, Stage Ⅲb, followed by adjuvant therapy with 8 courses CAPOX 2 years ago, had enhanced- computed tomography(CT)for follow-up and a 15-mm nodule near anastomotic site was found. 18F-fluorodeoxyglucose (FDG)-positron emission tomography(PET)CT revealed abnormal accumulation of 18F-FDG only to the lesion and diagnosis of"anastomotic recurrence"was made. We planned and safely performed resection of the anastomotic site and the nodule. The pathological diagnosis was fibromatosis-like tumor without evidence of recurrence, and margin was negative. Postoperative course was smooth and she was discharged on postoperative day 9. When we diagnose local recurrence, we need to keep it in mind that fibromatosis is one of the differential diagnoses, although its incidence rate is low.

[Surgical treatment of chondromyxoid fibroma of ribs via thoracoabdominal access]. / Khirurgicheskoe lechenie khondromiksoidnoi fibromy reber iz kombinirovannogo torakoabdominal'nogo dostupa.

Surgical treatment of chondromyxoid fibroma of ribs is described. The diagnosis was verified after histological analysis. The patient underwent resection of multinodular tumor of anterolateral thoracic wall invading abdominal cavity via thoracoabdominal access. Postoperative period was uneventful. This case demonstrates the need for total en-bloc resection of tumor with surrounding tissues. Surgery is the only effective method for these patients.

Papillary thyroid carcinoma with fibromatosis-like stroma: a case report and review of the literature.

BACKGROUND: Papillary thyroid carcinoma (PTC) is a common neoplasia with multiple variants. One of these extremely rare and poorly described variants is PTC with fibromatosis-like stroma (PTC-FMS), a peculiar entity distinguished by its predominant mesenchymal component. This paper reviews the literature, discusses the diagnostic challenges, and the clinical and surgical implications of this type of tumor which has fewer than 30 cases reported in the literature. CASE PRESENTATION: We reported a case of PTC-FMS found in a 41-year-old Italian woman, who came to our Institute with a recent growth in the form of a mass on the neck. Further immunohistochemical examination showed ß-catenin aberrant staining both in the nuclei and cytoplasm of the mesenchymal cells. The patient underwent total thyroidectomy and received radioactive iodine (RAI) 2 months after surgery. CONCLUSION: Given the possibility of recurrence of PTC-FMS and the ineffectiveness of RAI therapy, complete surgical resection represents the main treatment for this type of tumor. Despite the fact that the specific nature of these lesions has yet to be determined, guidelines for classical PTC should be followed.

Elastofibroma Dorsi: Case Series of a Rare Chest Wall Pseudotumor.

BACKGROUND: Elastofibroma dorsi (EFD) is a pseudotumor of the thoracic wall that can be difficult to diagnose due to its rarity. Prompt recognition can limit unnecessary workup and expedite treatment. This study retrospectively analyzed patients with a diagnosis of EFD, discussing clinical presentations and surgical outcomes. METHODS: This is an IRB-approved single-center retrospective study of all patients with a diagnosis of elastofibroma at our institution between 2000 and 2022. RESULTS: Ten patients were identified to have a pathologic diagnosis of EFD since 2000, with half presenting in the last 5 years. Our cohort had an average age of 56.8 years and was 50% female. The average age of male subjects was younger than females, 49.6-64.0 years, respectively (p = 0.10). Eighty percent (8/10) of patients had unilateral EFDs and symptoms lasted 27.1 months on average prior to diagnosis. Surgical resection was performed on 66.67% (8/12) of masses, with 87.5% (7/8) of patients who underwent surgery reporting complete resolution of their symptoms and none reporting recurrence. CONCLUSIONS: Although EFD is a rare pseudotumor, its incidence may be increasing. As such, surgeons should be aware of the typical clinical presentation; specifically, a slow growing, predominantly unilateral, painful, subscapular mass with an inhomogeneous pattern on imaging. Originally thought to predominantly affect elderly women, our study shows that younger men may be at risk as well. If patients present with EFD, complete surgical resection should be performed to achieve favorable outcomes and resolution of symptoms.

Nonossifying fibroma of the lower femur with genu valgum: a case report.

BACKGROUND: Nonossifying fibroma is common in children and adolescents, and nonossifying fibroma with genu valgum is rare in the clinic. This article evaluated the effectiveness of treatment in a case of nonossifying fibroma of the lower femur with genu valgum. CASE PRESENTATION: A 16-year-old girl complained of pain in the lower part of her right thigh for one year. She was diagnosed as non ossifying fibroma of the right femur with secondary valgus deformity of the right knee, and was treated in our hospital. We performed curettage, bone grafting and internal fixation,and corrected the valgum deformity at the same time. The patient's incision healed well, the pain was disappeared, and the mechanical axis of lower limbs was corrected. No tumor recurrence was found on X- ray examination one year after operation, and the fracture end was healed. The patient could walk normally, and she was satisfied with her limb function. CONCLUSION: Nonossifying fibroma with genu valgum is rare in the clinic. The patient was satisfied with our treatment, which achieved a good curative effect.

Concurrent medulloblastoma and cardiac fibroma: a rare presentation of Gorlin-Goltz syndrome.

BACKGROUND: Gorlin-Goltz syndrome is a rare autosomal dominant disorder resulting from PTCH1 gene mutation and presents with variable clinical manifestations. The co-occurrence of medulloblastoma and cardiac fibroma in Gorlin-Goltz syndrome is extremely rare. The present article discusses a patient diagnosed with Gorlin-Goltz syndrome and concurrent medulloblastoma and cardiac fibroma. CASE PRESENTATION: A 19-month-old boy transferred to our hospital after a radiological finding of posterior fossa lesion and hydrocephalus. A pericardial mass was noted after persistent arrhythmias. Both tumors were excised for definitive management. The histopathological sections were diagnostic of desmoplastic nodular medulloblastoma, WHO grade 4 and cardiac fibroma. Molecular and genetic investigations confirmed a pathogenic variant of PTCH1 gene, suggestive of autosomal dominant Gorlin-Goltz syndrome. CONCLUSION: Co-occurrence of medulloblastoma and cardiac fibroma is extremely rare and poses a management dilemma. Genetic counseling and antenatal screening are of utmost importance to early detect and manage patients with Gorlin-Goltz syndrome.

Lipofibromatosis-like neural tumors: Report of a case and review of 73 reported cases.

Lipofibromatosis-like neural tumors (LPF-NTs) are a recently discovered group of spindle cell tumors defined by the presence of a lipofibromatosis-like pattern, CD34 and/or S100 reactivity, and frequent neurotrophic receptor tyrosine kinase 1 (NTRK1) gene rearrangements. As new cases emerge, the spectrum of features observed in LPF-NTs continues to evolve. Here we describe the case of an 11-year-old with LPF-NT with a dermatofibrosarcoma protuberans-like honeycomb pattern, CD34 and S100 co-expression, and an NTRK1 rearrangement. We also review the clinical and molecular features of the 73 cases of LPF-NT previously described in the literature.

Ultrasonography of fibroma of the tendon sheath in the hand and wrist.

BACKGROUND: The purpose of our study was to explore the sonographic characteristics of fibromas of the tendon sheath of the hand and wrist and to evaluate the value of high frequency ultrasound in the diagnosis of FTS. METHODS: We retrospectively reviewed the sonography of 42 patients with surgically proven FTS, including one with a relapsing tumor (43 lesions in total). The location, size, distribution, relationship with the surrounding tissue, two-dimensional gray-scale sonographic appearance and internal color blood flow of all lesions were analysed. RESULTS: The maximum diameter ranged from 0.4 to 2.8 cm, with an average of 1.5 ± 0.6 cm. Twenty-eight lesions (65%) were associated with an adjacent tendon, while the other 15 lesions (35%) were next to the joint. Spindle or oval lesions were common, followed by irregular shape. The nodules with clear boundaries were hypoechoic and had posterior echo enhancement. Thirty-seven lesions (86%) were homogeneous, while 6 lesions (14%) had cystic components with no echo inside. Seventeen lesions (40%) had a large amount of blood flow. Nine lesions (20%) had a small amount of blood flow. The other 17 lesions (40%) had no significant blood flow. CONCLUSIONS: The diagnosis of fibroma of the tendon sheath can be considered when ultrasound examination reveals a focal nodular mass adjacent to a tendon sheath with homogeneous hypoechogenicity and no or small or large amounts of blood flow.

Recurrent chondromyxoid fibroma of the distal femur treated with percutaneous cryoablation.

Chondromyxoid fibroma is a rare, benign tumor of the bone with excellent prognosis but a high rate of recurrence. We report a patient presenting with pain and a history of chondromyxoid fibroma of the distal left femur previously treated with multiple prior curettage and bone graft procedures. Magnetic resonance imaging and histopathology indicated a recurrence of tumor. Due to the small size of the tumor recurrence and challenges associated with prior open surgery, the patient underwent cryoablation of the lesion with computed tomography guidance. Follow-up 18 months later indicated a resolution of pain and improvement on magnetic resonance imaging, and no concerns after 20 months. To our knowledge, this is the first reported case of chondromyxoid fibroma treated with cryoablation. This case suggests cryoablation could be considered in the setting of recurrent chondromyxoid fibroma for local tumor control.

A rare case of papillary fibroelastoma involving the tricuspid valve. A single center experience over a period of 22 years (1999-2021).

BACKGROUND AND AIM: Papillary fibroelastoma (PFE) represents only 16% of the benign cardiac tumor and approximately 15% of these are located on the tricuspid valve. MATERIALS AND METHODS: Over a period of 22 years (1999-2021) we observed 75 pts with cardiac tumors at our Center over 9650 pts operated on but only one case of a tricuspid valve PFE in a 69-year-old patient. Trans-thoracic echocardiography demonstrated a mobile mass (20 × 10 mm), adhering to the atrial side of the septal leaflet of the tricuspid valve of unknown origin. In consideration of the mobility of the mass and the consequent high embolic risk, surgical removal was made. The patient underwent surgery through a median sternotomy on CPBP. A 'gelatinous' mass adhering to the tricuspid leaflet was found and completely removed. The postoperative course was uneventful. The pathological diagnosis was PFE. CONCLUSIONS: PFEs of the tricuspid valve are rare entities being in most cases found incidentally. In our experience, the incidence of this tumor in this location is 1/10,000 cases of cardiac surgery. Although most patients are asymptomatic, surgical treatment is nevertheless recommended in consideration of the high embolic risk.

Prominent osteolysis in the maxilla: case report of an odontogenic fibroma mimicking a cyst.

BACKGROUND: Odontogenic fibroma (OF) is a rare benign odontogenic tumor of ectomesenchymal origin, mostly affecting the tooth-bearing portions of the jaws in middle-aged patients. Whilst small lesions tend to be clinically asymptomatic, varying unspecific clinical symptoms occur with an increase in size and may mimic odontogenic or other maxillofacial bone tumors, cysts, or fibro-osseous lesions of the jaws. CASE PRESENTATION: A 31-year-old female patient presented with a hard, non-fluctuating protrusion in the vestibule of the upper right maxilla. It was visualized on cone beam computed tomography (CBCT) as space-occupying osteolysis with the displacement of the floor and facial wall of the maxillary sinus, mimicking a cyst-like lesion. The tissue was surgically removed and identified as an OF in the histopathological examination. One year after the surgery, restitution of regular sinus anatomy and physiological intraoral findings were observed. CONCLUSIONS: This case report emphasizes that rare entities, like the maxillary OF presented, often demonstrate nonspecific clinical and radiological findings. Nevertheless, clinicians need to consider rare entities as possible differential diagnoses and plan the treatment accordingly. Histopathological examination is essential to conclude the diagnosis. OF rarely recur after proper enucleation.

[Acute Coronary Syndrome Caused by Papillary Fibroelastoma:Report of a Case].

A 72-year-old female had persistent severe chest pain while climbing stairs. She was diagnosed as having acute coronary syndrome, and underwent an emergency coronary angiography (CAG). The right coronary artery (RCA) ostium was sub-totally occluded. Echocardiography revealed a 10 mm mobile mass at the right coronary cusp of the aortic valve. To avoid total obustruction on two drug eluting stents were placed at the RCA ostium so as to have the proximal end protrude into the right Valsalva sinus. Thus, her hemodynamic condition was stabilized. The tumor was surgically resected and the stents were easily removed. Pathologically, the tumor was papillary fibroelastoma. Postoperative aortic regurgitation was minimal echocardiography, and CAG showed normal RCA.

A Recurrent Case of Ameloblastic Fibroma in 37-year Old Male.

Ameloblastic fibroma (AF) is a benign mixed epithelial and mesenchymal odontogenic tumor. This was previously grouped in odontogenic tumor showing odontogenic epithelium with odontogenic ectomesenchyme, with or without hard tissue formation. This report describes a case of ameloblastic fibroma in a 37-yearold male who came with the complain of swelling in the left side of lower jaw since one year. Enucleation of the mass followed by reconstruction was done six years back. However, after two years of initial treatment; radiographic findings suggested recurrence. Histopathological examination confirmed the diagnosis of ameloblastic fibroma. Patient had no clinical and radiographic evidence of recurrence in three and six months' follow-up. Because of the higher proliferative capacity and malignant degree of the mesenchymal component in the recurrent neoplasm, sarcomatous transformation may occur. Hence, a long term clinical and radiographical follow-up is essential due to its transformation into ameloblastic fibrosarcoma.

Giant dorsal lipofibromatosis in an infant: a case report.

BACKGROUND: Lipofibromatosis is a rare, benign, soft tissue tumor that usually presents in children. Low incidence and lack of specificity in clinical presentation make its diagnosis difficult. CASE PRESENTATION: This is a case report of a patient with a giant lipofibromatosis on the back that resembles an infantile hemangioma, which posed great difficulty in diagnosis due to atypical clinical manifestations. After the postoperative pathological and immunohistochemical examination and fluorescence in situ hybridization, the patient was finally diagnosed with lipofibromatosis. CONCLUSIONS: The incidence of fibromatosis was low. This case presents an atypical clinical manifestation since the tumor growth was on the back, and this can easily cause misdiagnosis. This case suggests that the diagnosis of lipofibromatosis depends on the pathology and fluorescence in situ hybridization.

A study of 399 cardiac tumors: Characteristics of echocardiography and pathological features.

AIMS: This study aimed to summarize the transthoracic echocardiography (TTE) characteristics of cardiac tumors with different pathologies. METHODS: The data of 399 patients with cardiac tumors confirmed by pathology, who had undergone surgical resection were consecutively collected in our hospital between January 1, 2011 and December 31, 2019. The TTE characteristics were summarized and compared with the pathology. RESULTS: Mean patient age was 49.8±15.7 years (22 children and 377 adults), and 62.2% were female. Of the tumors, 90.5% (361) were primary and 9.5% (38) were secondary. Further, 88.7% (354) were benign and 11.3% (45) were malignant. Of the primary tumors (96.1% benign and 3.9% malignant), 84.2% were myxomas, followed by 3.5% lipomas and 1.5% fibromas in adults, while in children, 31.8% were rhabdomyomas and 22.7% were fibromas. The most common type of secondary cardiac tumor was malignant liver carcinoma metastasis (39.5%) and benign intravenous leiomyomatosis with cardiac extension from the uterus (18.4%). TTE features of myxoma showed four variation types among 8.9% of myxomas: liquefaction (anechoic region mostly), calcification (hyperechoic range with a shadow), multiple nodules, and high proliferative activity (a large irregular mass with a wide base and a high Ki67 index). The TTE characteristics of some common benign non-myxoma tumors had specific findings. The TTE features of malignant tumors mostly showed hypoechogenicity, an unclear boundary, a wide basement, and multi-chambers or tissue invasion. CONCLUSIONS: Most cardiac tumors have typical ultrasonic manifestations. Preoperative echocardiography could roughly judge cardiac tumor type and may be helpful for guiding clinical treatment decisions.