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Phosphaturic mesenchymal tumor (PMT) is a rare neoplasm that causes tumor-induced osteomalasia (TIO) in most affected patients, usually through the production of fibroblast growth factor 23 (FGF23). This tumor is often misdiagnosed due to its relative rarity and its widely varied histomorphologic spectrum. Here we describe a case of a 78-year-old woman who presented with a left middle tumor without symptoms of TIO. The histological features resembled chondromyxoid fibroma with smudgy calcification in the tumor matrix. In addition, we evaluated FGF23 expression through immunohistochemical study and reverse transcription polymerase chain reaction. PMT with chondromyxoid fibroma features are extremely rare. Examining the expression of FGF23 is useful in the diagnosis of PMT., (© 2023 Japanese Dermatological Association.)

Papillary fibroelastoma is an uncommon primary cardiac tumor, predominantly associated with left-sided valvular endocardium. Affectation of tricuspid valve leaflets is rare, and management in asymptomatic patients remains controversial. We present a 30-year-old female referred for evaluation prior to bariatric surgery. A routine echocardiogram revealed a mobile cardiac mass attached to the tricuspid valve. A patent foramen ovale was also present. Tumor was surgically removed. Histology confirmed the definite diagnosis of a papillary fibroelastoma. In our case, the presence of a patent foramen ovale associated with the right-sided fibroelastoma was decisive in the decision for surgery., (© 2017, Wiley Periodicals, Inc.)

Introduction: Papillary fibroelastoma (PFE) is a benign cardiac tumor that is currently detected more often due to the technological improvements in echocardiography., Objectives: To describe the echocardiographic features of PFE and correlate them with the clinical presentation and initial treatment., Materials and Methods: A prospective analysis of patients with a diagnosis of PFE was conducted between 2000 and 2015. We assessed the clinical history, symptoms at the time of diagnosis, echocardiographic features, and initial treatment., Results: Fifty-four patients with a diagnosis of PFE by echocardiography were included. The incidence was 0.038%. Mean age was 62±16 years; 50% were male. Forty-six percent of patients had symptoms at the time of diagnosis, the most frequent of which was transient ischemic accident (TIA). Embolic episodes occurred in 31% of patients, mainly to the brain. PFEs were valvular in 70.4% of cases and multiple in 13%. Mean maximum length was 1.18±0.58 cm, and 44% were mobile. PFEs >1.5 cm were most often found in the heart valves (56.8% vs 10.8%; P=.045). There was no significant relation between size, mobility, location and number of tumors, and the presence of embolism or symptoms. Most frequent treatment was oral anticoagulation in 48.6%, followed by simple tumor resection in 42.8% of cases., Conclusions: PFE is a small tumor, predominantly valvular and benign, but entails a high incidence of cerebral embolism. The initial approach should be individualized according to clinical manifestations, comorbidities, and the experience of the surgical center., (© 2016, Wiley Periodicals, Inc.)

Background: Soft tissue perineurioma (STP) is a benign peripheral nerve sheath tumor demonstrating uniform perineurial cell differentiation. To the authors' knowledge, the cytomorphologic features of STP remain incompletely characterized, and the distinction between STP and its benign (intramuscular/cellular myxoma) and malignant (low-grade fibromyxoid sarcoma [LGFMS]) mimics is challenging., Methods: Fine-needle aspiration (FNA)/core needle biopsies of 25 low-grade myxoid spindle cell neoplasm cases including STP (5 cases), intramuscular/cellular myxoma (16 cases), and LGFMS (4 cases) were reviewed retrospectively for cytomorphologic and immunophenotypic comparison., Results: FNA smears of STP were hypocellular with scattered clusters of spindle cells with bland, slender nuclei; bipolar cytoplasmic processes; and scant myxoid to collagenous matrix. STP commonly lacked the abundant granular myxoid matrix material present in intramuscular/cellular myxoma (20% in STP vs 75% in intramuscular/cellular myxoma; P <.05), but these tumors were otherwise remarkably found to be similar on FNA smears. All STP and intramuscular/cellular myxoma cases lacked cytologic atypia, whereas 50% of LGFMS cases demonstrated mild nuclear atypia. EMA was positive in all STPs, but also was found to be at least focally positive in 60% of intramuscular/cellular myxoma cases (9 of 15 cases) and 75% of LGFMS cases (3 of 4 cases). MUC4 was found to be negative in all 15 intramuscular/cellular myxoma and 5 STP cases, but was positive in all 4 LGFMS cases., Conclusions: STP, intramuscular/cellular myxoma, and LGFMS have significant cytomorphologic overlap. Immunohistochemical staining with EMA is not beneficial due to a lack of specificity. Negative MUC4 staining reliably excludes LGFMS. Therefore, a clinically meaningful approach to the FNA biopsy evaluation of a low-grade myxoid spindle cell neoplasm is to provide a differential diagnosis and to exclude a low-grade sarcoma. Cancer Cytopathol 2016;124:651-8. © 2016 American Cancer Society., (© 2016 American Cancer Society.)

Background: Cardiac fibromas are primarily detected in infants and children but are extremely rare in adults., Methods: We report a case of a cardiac fibroma in a 37 year old male and review the literature on this subject., Result: The patient was referred for evaluation of a cardiac mass. Echocardiography and computed tomography examinations revealed it as an inhomogeneous mass with calcifications. Subsequently, the lesion was completely excised and confirmed to be a cardiac fibroma. Cardiac fibromas account for only a very small percentage of all cardiac tumors. The occurrence and severity of symptoms caused by cardiac fibromas are primarily determined by the sites and size of lesions. Imaging techniques are very sensitive in diagnosing cardiac fibromas, defining the extent and planning surgical approach. Surgical treatment of cardiac fibromas gives excellent early and late survival., Conclusion: Although cardiac fibromas are benign, their behavior is unpredictable. Surgery appears to be the optimal treatment for patients with resectable tumors., (© 2013 Wiley Periodicals, Inc.)

Background: Ovarian fibromas/fibrothecomas are uncommon benign tumours of ovary. Due to their solid structure, these benign tumours are sometimes confused with malignant tumours during clinical evaluation., Aims: To determine the clinico-pathological characteristics of ovarian fibroma/fibrothecoma and analyse the efficiency of risk of malignancy index (RMI) scoring system to distinguish malignancy among these tumours., Methods: Between November 2001 and February 2012, women with a pathological diagnosis of ovarian fibroma/fibrothecoma were identified. Depending on the menopausal status, serum CA-125 level and ultrasonographic findings, RMI scores were calculated for each of the patients., Results: During the study period, 43 ovarian fibroma/fibrothecoma (4.7%) were detected among 912 adnexal masses operated. The mean age of the women was 52.2 (range, 21-80 years). Upon calculating RMI scores, 33 women (76.7%) were classified as low risk and 10 women (23.3%) as high risk for malignancy. Sensitivity, specificity, positive predictive value and negative predictive value of the RMI scoring for identification of malignant lesions preoperatively were found as 0%, 76%, 0% and 97%, respectively. Final pathological diagnosis was ovarian fibroma in 13 (30%) women, fibrothecoma in 29 (67%) and fibrosarcoma in one woman (2%)., Conclusion: There are no specific markers for accurate preoperative diagnosis of ovarian fibroma/fibrothecoma. Moreover, according to our results, RMI scoring system does not aid clinicians in this issue either, with a high false-positive rate and very low sensitivity. Further studies with higher number of cases are needed to state clearly the role of RMI scores in preoperative discrimination of malignancy., (© 2013 The Authors ANZJOG © 2013 The Royal Australian and New Zealand College of Obstetricians and Gynaecologists.)

A young patient underwent a screening electrocardiogram (EKG) that suggested apical hypertrophic cardiomyopathy. Serial investigation with echocardiography showed a well-defined hyperechogenic mass involving the interventricular septal. To better define the lesion extension three-dimensional (3D) echocardiography was done and it demonstrated a mass invading the septal myocardium, involving the major part of the muscular portion. The findings were highly suggestive of a cardiac fibroma. A cardiac magetic resonance image (MRI) was also compatible with this diagnosis. In our case, 3D echo showed a high accuracy, proving to be a useful tool to determine the anatomy of the lesion, complementary to MRI, guiding best management strategy., (© 2013, Wiley Periodicals, Inc.)

We report a case of a 54-year-old female with papillary fibroelastoma of the aortic valve who presented with ST-elevation myocardial infarction and cardiac arrest. Though her initial symptom was only atypical chest pain, life-threatening complications such as acute myocardial infarction and cardiac arrest developed. After cardiopulmonary resuscitation, we promptly resected the fibroelastoma on cardiopulmonary bypass, and the patient was discharged without any other complications., (© 2012 Wiley Periodicals, Inc.)

Papillary fibroelastoma (PFE) is a benign primary tumor of the heart usually originating from the heart valves. Nonvalvular fibroelastomas are rare, and reported cases have presented either incidentally or with cerebral embolic phenomena; none have reported recurrent anginal symptoms. We are reporting a case of a 53-year-old female with history of significant radiation exposure to the chest in the past, who presented with recurrent chest pain and was found to have left atrial nonvalvular PFE managed with surgical excision. , (© 2011 Wiley Periodicals, Inc.)

Complex aortic valve repair after mass lesion resection, in an otherwise normal, thin leafleted valve, is rarely described in the literature. We present a 68-year-old woman who underwent resection of an asymptomatic aortic valve papillary fibroelastoma. Due to extensive involvement of her left coronary cusp, the resection resulted in a significant defect in the leaflet, requiring a complex repair to preserve her otherwise normal aortic valve. We describe the operative findings, repair technique, and associated literature., (© 2010 Wiley Periodicals, Inc.)

Nonmyxomatous tumors of the heart are exceedingly rare in adults. We report a case of symptomatic, massive, right ventricular cardiac fibroma found in an adult. Despite multiple (preoperative) biopsies, diagnosis was made only after surgical excision., (© 2010 Wiley Periodicals, Inc.)

Cardiac fibromas are rare benign tumors predominantly diagnosed in children and teenagers. We report a 57-year-old male patient with class II dyspnea and angina. During electrocardiographic assessment, recurrent ventricular tachycardia was present and magnetic resonance imaging revealed a giant (12.5 × 5 × 4 cm) intramural left ventricular tumor compatible with cardiac fibroma. After surgical biopsy and histological confirmation, open-heart surgical resection was successfully performed. After nine months of follow up, the patient remains asymptomatic without evidence of ventricular tachycardia., (© 2010 Wiley Periodicals, Inc.)

Cardiac papillary fibroelastoma is a benign tumor that mainly affects cardiac valves. The tumor has the potential to cause angina and myocardial infarction due to embolization of tumor fragments. We describe a rare case of right coronary artery ostial obstruction by a 12 x 19 mm sized papillary fibroelastoma located in the sinus of Valsalva. The report underlies the importance of echocardiography in diagnosis and intraoperative treatment of this type of cardiac mass.

Chondromyxoid fibroma of the skull base is a rare entity. Involvement of the temporal bone is particularly rare. We present an unusual case of progressive facial nerve paralysis with imaging and clinical findings most suggestive of a facial nerve schwannoma. The lesion was tubular in appearance, expanded the mastoid facial nerve canal, protruded out of the stylomastoid foramen, and enhanced homogeneously. The only unusual imaging feature was minor calcification within the tumor. Surgery revealed an irregular, cystic lesion. Pathology diagnosed a chondromyxoid fibroma involving the mastoid portion of the facial nerve canal, destroying the facial nerve.

A 76-year-old male was admitted with increasing fatigue and shortness of breath. Transesophageal echocardiogram revealed moderate-to-severe mitral regurgitation and an oscillating mass on the pulmonic valve that was confirmed by magnetic resonance imaging. The patient underwent repair of the mitral valve and excision of the pulmonic valve mass, which was confirmed as a papillary fibroelastoma by histology.

In this study we investigated the accuracy of diagnoses of oral mucosal diseases made by family physicians (without a dental degree), other categories of physicians, and general dental practitioners prior to referring patients to a university oral medicine unit. Over a three-year period, we compared the diagnoses proposed in referral letters with the definitive histological diagnoses made by the specialist unit. Only 305 of 678 (45 percent) of the referral letters included a clinical diagnosis. Eighty-six patients (86/305, 28 percent) were referred by general dental practitioners (GDPs) who had graduated in dentistry; seventy-six (76/305, 25 percent) were sent by GDPs who had graduated in medicine with a postgraduate degree in dentistry; and 143 (143/305, 47 percent) were referred by other categories of physicians. More than 50 percent of the referring professionals were not able to make a clinical diagnosis of oral mucosal diseases. Only 40 percent of the provisional diagnoses (122/305) coincided with the diagnosis made at the specialist unit. The proportion of correct diagnoses was 40 percent for GDPs who had graduated in dentistry, 33 percent for other categories of physicians, and 27 percent for GDPs who had graduated in medicine with a postgraduate degree in dentistry. These findings suggest that Italian dental and medical practitioners have limited knowledge in the field of oral medicine. Consequently, there is a need for better education in the diagnosis and treatment of oral diseases and for improvement in total oral health training.

Cardiac papillary fibroelastomas (CPF) are benign endocardial papillomas commonly formed from valve endothelium. The majority of tumors are found on the left side of the heart, with only a few case reports of pulmonary valve fibroelastomas. We report here a case of pulmonary valve papillary fibroelastoma that was successfully managed with simple excision of the mass.

Cardiac papillary fibroelastoma (CPF) is a histologically benign tumor that can cause a significant embolic event. CPF is usually located on the endocardium, and is found on valves in 90% of cases. We experienced an unusual case of CPF situated on a left ventricular anterolateral papillary muscle. An 84-year-old female who had developed cerebellum infarction five months previously underwent surgical resection of CPF. During surgical resection of the tumor, a fiberscope was useful for detecting the lesion, and allowed us to ensure complete resection with a good surgical view. Our experience encourages us to use the fiberscope when resecting other intracardiac masses, including thrombi.

Primary cardiac tumors are rare, with benign lesions outnumbering malignant ones by a margin of over four to one. Amongst the benign cardiac neoplasms, papillary fibroelastomas are reported second only to myxomas. Papillary fibroelastomas commonly rise from heart valves and their size ranges from a few millimeters to well over 2.0 cm in diameter. While myxomas commonly arise from the interatrial septum, we present the rare example of a large papillary fibroelastoma arising from the left side of the interatrial septum.

Papillary fibroelastomas are uncommon benign tumors usually involving heart valves that may be potential sources of emboli. Transesophageal echocardiography has greatly enhanced the ability to make the diagnosis of these surgically treatable tumors in a timely fashion. We describe an unusual presentation of a 62-year-old man with suspected bacterial endocarditis, in whom initial transesophageal echocardiogram suggested the presence of pacemaker lead infection but a repeat study after extraction of the pacemaker lead revealed a large pedunculated mass arising from the superior vena cava. The mass was surgically removed and histopathology revealed papillary fibroelastoma. This case is unusual with respect to the size and site of origin of the papillary fibroelastoma as well as its echocardiographic presentation mimicking vegetations on a pacemaker wire.

This article describes four patients with non-ossifying fibromas (NOFs) and multiple café-au-lait spots. Two of the patients were diagnosed with NOFs when they presented with a femur fracture. The other two patients were diagnosed with NOFs because of complaints of leg problems. In addition, axillary freckles and Lisch nodules were present in all four patients and multiple cutaneous neurofibromas in two patients. These four patients fulfilled the diagnostic criteria for neurofibromatosis type 1 (NF1) and also have been diagnosed with Jaffe-Campanacci syndrome. We propose that Jaffe-Campanacci syndrome is a manifestation of NF1 and suggest that patients with NF1 should have more rigorous radiographic screening of the long bones during early adolescence or adulthood to determine the presence or absence of NOFs. Appropriate intervention (exercise restriction, bracing, and/or surgery) might decrease the long-term disability associated with Jaffe-Campanacci syndrome., (Copyright 2003 Wiley-Liss, Inc.)

A 16-year-old boy presented with painless swellings localized to the radial and ulnar aspects of his second through to the fifth fingers on the left hand, with more subtle changes affecting two fingers on the opposite hand. This had developed in the absence of mechanical trauma. Investigations for an arthropathy were negative, while a biopsy showed marked epidermal hyperplasia and an expanded dermis. These features are typical of pachydermodactyly, a benign dermatosis of uncertain aetiology. The interesting feature of this case is the presence of knuckle pads in the father of the patient, which raises the possibility that these two similar entities are related.

Primary tumors of the heart are rare, with an incidence between 0.001% and 0.33% in autopsy findings, and with fibromas representing about 4% of benign cardiac tumors. We report the case of a cardiac fibroma in a 5-year-old child affected by Sotos syndrome. The mean sign was a ventricular tachycardia.

Perifollicular fibroma(PFF) is a rare cutaneous hamartoma that shows differentiation in the connective tissue sheath of the hair follicle. It may be single (congenital or acquired) or multiple (late onset). We report a 14-year-old Korean boy with a congenital solitary PFF on the face, which supports the nevoid concept of origin rather than a reactive response to injury.

A patient presented with a solitary large subcutaneous tumor homogeneously composed of loose fibroblasts interspersed with abundant mucinous material. The literature was reviewed, and the origin, pathogenesis and clinical course of this rare neoplasm were briefly discussed.

We reported a fourteen-month-old boy with infantile digital fibromatosis. At the age of seven months, a nodule appeared on the back of the left third toe, and developed into a slight red tumor divided into five hemispherical nodules. Histopathologically, spindle-shaped tumor cells with an eosinophilic inclusion body in the cytoplasm were seen in the dermis. Electron microscopy showed a dense body in the cytoplasm of the tumor cells. One year and two months after the first visit, the tumor regressed without any aggressive treatment. Japanese cases of infantile digital fibromatosis were reviewed. The literature review and our case suggest that the tumor should be observed without any aggressive treatment unless it causes mobile dysfunction of the affected finger or toe.

The following case report is of a 49 year old man who presented with a 20 year history of small asymptomatic flesh coloured firm papules which were concentrated on his face, neck and upper trunk. The diagnosis of multiple fibrofolliculomas of the familial type was reached.

A comparative assessment was made of dermatofibroma, dermatofibrosarcoma protuberans, and malignant fibrous histiocytoma. The study was comprised of an immunohistochemical investigation using the Ki-67 monoclonal antibody and a quantitative analysis of cell nuclear DNA using flow cytometry. It was found that the index of Ki-67 monoclonal antibody labeling, the histogram pattern, and DNA index correlated with clinical malignancy, suggesting the usefulness of these indices for differential diagnosis and evaluation of prognosis.

In the past laryngeal pseudosarcomas have been diagnosed as a. carcinosarcomas, b. pleomorphic or spindle cell carcinomas, or c. squamous cell carcinomas with pseudosarcomatous reactive stroma. Arguments have centered around the nature of the sarcomatous stroma. Because of this confusion there is disagreement as to the treatment and prognosis of these tumors. Seven pseudosarcomas were treated between 1969-1979, 4 were pedunculated and 3 exophytic. Treatment consisted of primary CO60 irradiation in 2 patients, surgery in 3 cases and combined therapy in 2 cases with no recurrences. Three of the 7 have died, 1 of a poorly differentiated adenosquamous carcinoma of the right main stem bronchus and the other 2 of natural causes at ages 77 and 85. From a review of the literature as well as our experience, we have reached the following conclusions. 1. Stromal cells are a malignant morphologic variant of the squamous cell and are best termed spindled cells. 2. Neck metastasis at any time is a poor prognostic sign. 3. The pattern of metastasis and survival seems to parallel laryngeal squamous cell carcinoma, and thus treatment should be similar for given stages.

Four unusual cases of pseudotumor, which had clinical and radiographic findings suggesting neoplasia, are presented. One involved the maxillary sinus, destroying the lateral wall, and extending to the infratemporal fossa and the orbit. The second presented as an enlarging lacrimal gland tumor with ipsilateral parotid adenopathy, and the third as a large parapharyngeal mass that was diagnosed as a lymphoma on aspiration cytology. The fourth case documented the rare occurrence of a congenital pseudotumor, manifesting as proptosis in a neonate and thought to be a fibrosarcoma on frozen section study. Aggressive pseudotumors must be distinguished from malignancy to avoid unnecessary surgery or irradiation. Some cases can be distinguished from neoplasms by their dramatic response to oral steroid therapy, and others by characteristic features on CT or MRI. Due to the diverse cellular proliferation, aspiration cytology is infrequently helpful and diagnosis can be difficult on frozen section study. An approach to the diagnosis and management of pseudotumors is detailed.