Proliferative fasciitis: A rare cause of disturbances in an adolescent hand.

We report a case of hand soft tissue tumor-proliferative fasciitis (PF)-in a 12-year-old patient that presented as a painful lump causing trigger finger. After meticulous diagnostic workup, a surgical excision led to immediate amelioration of symptoms. PF is a rare benign pseudosarcomatous lesion arising typically in the subcutaneous tissue and fascia in adults. It is very uncommon in the hand. To the best of our knowledge, this is the first report of a trigger finger being caused due to this pathology. In this report, the authors review PF lesions on hands, advice careful evaluation of magnetic resonance imaging features, and recommend surgical management.

Papillary thyroid carcinoma with desmoid-type fibromatosis: A clinical, pathological, and immunohistochemical study of 14 cases.

Papillary thyroid carcinoma (PTC) with desmoid-type fibromatosis (DTF) is characterized by genetic alterations of the fibroblasts. PTC-DTF is extremely rare, and the reports on such cases have been sporadic. Immunohistochemical staining using the antibody for beta-catenin is useful in diagnosing the variant. This report aims to describe the clinical, pathological, and immunohistochemical findings in 14 cases of PTC-DTF and to clarify the diagnostic significance of the variant. The patients included 9 women and 5 men, with a mean age of 49.3 years. PTCs with focal DTF components and with extensive DTF components included 7 cases each. No significant differences were noted in terms of age, gender, and serum thyroglobulin levels between extensive and focal DTF cases. On aspiration cytology, 12 cases were reported as suspicious for malignancy or malignant, and schwannoma or fibroma was suggested in 1 case each. The DTF components were histologically classified into 4 types, namely, central (4 cases), peripheral (1 case), mixed (7 cases), and diffuse type (2 cases). The stromal components were consistent with those of DTF. Immunohistochemically, fibroblasts in the DTF components showed nuclear and cytoplasmic expression for beta-catenin in 12 cases. The features are observed even in cases in which stromal components focally exist. Neither carcinoma cells nor the fibroblasts with Ki-67 labeling index >5% were found in all cases. We agree that PTC with nodular fasciitis-like stroma should be renamed to PTC-DTF.

Biology and Treatment of Aggressive Fibromatosis or Desmoid Tumor.

Aggressive fibromatosis, also known as desmoid-type fibromatosis (DTF) or desmoid tumor, is an uncommon locally invasive tumor. Because of its low incidence and variable behavior, DTF is often first seen by physicians who are not familiar with it, and recent advances in understanding this disease have led to changes in treatment approaches. The Wnt (ß-catenin) pathway appears to play a key role in DTF pathogenesis, and recent studies of DTF biology suggest a possible model of DTF pathogenesis. Histologically, DTF shows a poorly circumscribed proliferation of myofibroblast-like cells with variable collagen deposition, similar to the proliferative phase of wound healing, and DTF has been associated with trauma and pregnancy. Desmoid-type fibromatosis may be a useful model of the tumor stroma in carcinomas as well as other fibrosing diseases such as progressive pulmonary fibrosis. The clinical course of DTF can vary greatly among patients, complicating the determination of the optimal treatment approach. Treatment options include surgery, nonsteroidal anti-inflammatory drugs with or without hormonal manipulation, chemotherapy, radiation therapy, and other forms of local therapy. Many treatments have been used, but these are not without toxicities. Because of the variable nature of the disease and the potential morbidity of treatment, some cases of DTF may do better without treatment; simple observation is often the best initial treatment. This review used a PubMed search from January 1, 1980, through October 31, 2016, using the terms fibromatosis and desmoid and discusses DTF disease characteristics, pathophysiology, and treatment options as well as examines several cases illustrating key points in the biology and treatment of this heterogeneous disease.

Duodenal plexiform fibromyxoma as a cause of obscure upper gastrointestinal bleeding: A case report.

RATIONALE: We are reporting the first-to our knowledge-case of duodenal Plexiform Fibromyxoma causing obscure upper gastrointestinal bleeding. PATIENT CONCERNS: Plexiform fibromyxoma triggered recurrent upper gastrointestinal bleeding episodes in a 63-year-old man who remained undiagnosed, despite multiple hospitalizations, extensive diagnostic workups and surgical interventions (including gastrectomies), for almost 17 years. DIAGNOSES-INTERVENTIONS: During hospitalization for the last bleeding episode, an upper gastrointestinal endoscopy revealed an intestinal hemorrhagic nodule. The lesion was deemed unresectable by endoscopic means. An abdominal computerized tomography disclosed no further lesions and surgery was decided. The lesion at operation was found near the edge of the duodenal stump and treated with pancreas-preserving duodenectomy (1st and 2nd portion). OUTCOMES: Postoperative recovery was mainly uneventful and a 20-month follow-up finds the patient in good health with no need for blood transfusions.Plexiform fibromyxomas stand for a rare and widely unknown mesenchymal entity. Despite the fact that they closely resemble other gastrointestinal tumors, they distinctly vary in clinical management as well as the histopathology. Clinical awareness and further research are compulsory to elucidate its clinical course and prognosis.

Elastofibroma dorsi: Clinical evaluation of 61 cases and review of the literature.

OBJECTIVE: Elastofibroma dorsi (ED) is a rare, benign, soft tissue tumor typically located between inferior corner of scapula and posterior chest wall causing mass, scapular snapping, and pain. When classic symptoms and localization are present, it is diagnosed without biopsy and treated with marginal resection. This study retrospectively analyzed patients operated on for ED to evaluate presenting symptoms, tumor size, complications, and clinical results, and to suggest optimal treatments. METHODS: This study included 51 patients who underwent surgery for ED in 2 different clinics between 2005 and 2015. Patient age, gender, profession, side affected, symptoms, average duration of symptoms, and tumor size were researched. Radiological examinations of patients were evaluated. Patients with lesions larger than 5 cm in size were operated on. Postoperative complications, recurrence, and functional results were evaluated using Constant score and compared to preoperative values. RESULTS: A total of 61 operated lesions of 51 patients clinically and radiologically diagnosed with ED were retrospectively evaluated. Average length of time patient experienced symptoms was 11.21 months. Lesions in 19 (37.2%) patients were bilateral, 10 of which were symptomatic and larger than 5 cm in size, meeting indication for surgery. Average lesion diameter was 8.7 cm. Average follow-up was 26.89 months. Average of preoperative Constant score of 67.28 subsequently increased to 92.88 (p < 0.05). Seroma and hematoma were observed in 11.5% of patients. CONCLUSION: Generally, good clinical results can be obtained with marginal resection without requiring a biopsy, considering classic complaints and radiological appearance of ED. LEVEL OF EVIDENCE: Level IV, Therapeutic study.

Pachydermodactyly: An Unknown Entity.

Pachydermodactyly is a rare benign disease that usually affects the dorsal skin and subcutaneous tissue of the proximal interphalangeal joints of the index, middle, ring, and little fingers; the thumb is usually spared. Hyperkeratosis on the dorsal side of the affected areas is common. We present a case of a 19-year-old man who was diagnosed with pachydermodactyly after evaluation of a painless, progressive swelling and hyperkeratosis at the proximal interphalangeal and distal interphalangeal joints of all fingers, bilaterally, for 6 years before presentation. Conservative treatment was prescribed and the patient remains asymptomatic. Given the benign and nonprogressive course of the disease, symptomatic treatment alone is all that is usually required.

Regional hyperthermia combined with chemotherapy in paediatric, adolescent and young adult patients: current and future perspectives.

Here we evaluate the current status of clinical research on regional hyperthermia (RHT) in combination with chemotherapy or radiation therapy in paediatric oncology.Data were identified in searches of MEDLINE, Current Contents, PubMed, and references from relevant articles using medical subject headings including hyperthermia, cancer, paediatric oncology, children, radiation therapy and chemotherapy. Currently, only two RHT centres exist in Europe which treat children. Clinical RHT research in paediatric oncology has as yet been limited to children with sarcomas and germ cell tumours that respond poorly to or recur after chemotherapy. RHT is a safe and effective treatment delivering local thermic effects, which may also stimulate immunological processes via heat-shock protein reactions. RHT is used chiefly in children and adolescents with sarcomas or germ cell tumours located in the abdomino-pelvic region, chest wall or extremities to improve operability or render the tumour operable. It could potentially be combined with radiation therapy in a post-operative R1 setting where more radical surgery is not possible or combined with chemotherapy instead of radiation therapy in cases where the necessary radiation dose is impossible to achieve or would have mutilating consequences. RHT might also be an option for chemotherapy intensification in the neoadjuvant first-line treatment setting for children and adolescents, as was recently reflected in the promising long-term outcome data in adults with high-risk soft tissue sarcomas (EORTC 62961/ESHO trial).The limited data available indicate that combining RHT with chemotherapy is a promising option to treat germ cell tumours and, potentially, sarcomas. RHT may also be beneficial in first-line therapy in children, adolescents and young adults. The research should focus on optimising necessary technical demands and then initiate several clinical trials incorporating RHT into interdisciplinary treatment of children, adolescents and young adults that include translational research components exploring potential immunological mechanisms of action.

Pachydermodactyly: A Case Report Including Histopathology.

Pachydermodactyly (PDD) is a rare form of digital fibromatosis characterized by painless soft tissue swelling, primarily about the proximal interphalangeal joints. The skin at the metacarpophalangeal joints, the palm, and the dorsum of the hand may also be involved. Because swelling can occur over the proximal interphalangeal and metacarpophalangeal joints, PDD may be confused with juvenile inflammatory arthropathy and may even occur concurrently. We present the clinical and histopathologic findings of a case of PDD characterized by bilateral proximal phalangeal involvement of the index through little fingers.

Fibromatosis of infratemporal space.

Fibromatosis is a rare benign mesenchymal neoplasm which primarily originates in the muscle, connective tissue, fascial sheaths, and musculoaponeurotic structures. It is commonly seen as abdominal tumour but in maxillofacial region, the occurrence of these tumours is very rare and exceedingly rare in infratemporal space. Often misdiagnosed due to its varied clinical behaviour, fibromatosis is benign, slow-growing, infiltrative tumour without any metastatic potential, but is locally aggressive causing organ dysfunction along with high recurrence rate. We report a case of fibromatosis involving the left infratemporal space in a 35-year-old female who presented with chief complaint of limited mouth opening for the preceding 4 years.

Pediatric cardiac tumors: a 45-year, single-institution review.

BACKGROUND: Cardiac tumors in children are rare. Of the cases reported in the literature, nearly all are benign and managed conservatively. METHODS: This is a retrospective, observational study of pediatric patients <18 years who presented for surgical evaluation of a cardiac tumor, between 1969 and 2014 at a tertiary care children's hospital. Presentation, pathology, management, and outcomes were evaluated. RESULTS: Over the last 45 years, 64 patients were evaluated for surgical resection of a cardiac tumor. Rhabdomyoma was the most common neoplasm (58%), and 17% of the tumors had malignant pathologies. While 42% of benign cardiac neoplasms required surgical intervention for significant hemodynamic concerns, 73% of malignant neoplasms underwent radical excision, if possible, followed by adjuvant chemotherapy. Despite a 37% mortality in patients with malignant pathology, an aggressive surgical approach can yield long-term survival in some patients. There were no deaths among patients with benign tumors and 17% had postoperative complications mostly related to mitral regurgitation. CONCLUSION: Cardiac tumors in children are rare but can be managed aggressively with good outcomes. Benign tumors have an excellent survival with most complications related to tumor location. Malignant tumors have a high mortality rate, but surgery and adjuvant chemotherapy allow for prolonged survival in selected patients.

Elastofibroma dorsi: a histochemical and immunohistochemical study.

Elastofibroma dorsi (ED) is considered a member of a heterogeneous group of benign fibrous (fibroblastic or myofibroblastic) soft-tissue tumors, frequently localized in the periscapular region in middle aged or older individuals. However, the pathogenesis of ED is still unclear and many authors believe that ED results from a reactive hyperproliferation of fibroblastic tissue, while others suggest that it may be a consequence of a mechanical friction. In our study, we examined 11 cases of ED using histochemical and immunohistochemical methods, in order to extend the knowledge about extracellular matrix composition and histopathogenesis of ED. From the results it appeared that stroma and interspersed spindle cells of ED were positive for both periostin and tenascin-C. Mast cells tryptase-positive were also abundant throughout the lesion. The perivascular distribution of periostin and tenascin-C, associated with the CD34 positivity, suggest that endothelial-mesenchymal transition events can account for neovascularization and production of fibroelastic tissue characteristic of elastofibroma. Our data obtained in endothelial cells cultures demonstrated that elastin production is higher when the status of confluence of the cells is low. So, we can assume that such a phenomenon is a characteristic of mesenchymal/endothelial cells CD34 positive, in which elastin production results to be inversely proportional to the vascular differentiation of cellular elements. In the light of these considerations, we think that a cancerous nature of ED is unlikely. Overall, our study report, for the first time, a detailed description of extracellular matrix composition in ED, suggesting that a mechanical strain-dependent reactivation of periostin and tenascin-C expression, as well as of elastin deposition, could be responsible for development of ED.

Papillary fibroelastoma of tricuspid valve presenting as pulmonary embolism.

A 65-year-old gentleman was admitted with recurrent dyspnea. Two-dimensional and transesophageal echocardiography revealed a highly mobile echogenic mass attached to the tricuspid valve. A (99m)Tc lung perfusion scan was suggestive of pulmonary embolism. The patient underwent surgical resection of the mass. Histopathological examination revealed a papillary fibroelastoma.

[Successful excision of a left ventricular fibroma in an adult patient; report of a case].

A 55-year-old man was referred with a diagnosis of a left ventricular thrombus. Echocardiography revealed that he had a thrombus in the free wall of the left ventricle. Under cardiopulmonary bypass, we removed the intramural tumor. After the removal, the defect was repaired by Dor operation. Pathological examination revealed the tumor was a cardiac fibroma. He is doing well without any troubles 3 years after the operation.

Multiple papillary fibroelastoma: report of a case and implications for management.

A 59-year-old woman with chest discomfort was transferred to our hospital. Echocardiography was suggestive of multiple papillary fibroelastoma (PFE). Tumors on both the left and right coronary cusps were confirmed macroscopically and pathologically and a small tumor was noted microscopically on the non-coronary cusp. Aortic valve replacement was successfully performed. The treatment and recurrence rate of PFE is controversial due to its rarity. Given that multiple tumors were seen in the present case and that possible recurrence has been reported elsewhere, valve replacement may be a better choice for surgical repair than valvoplasty in some cases, such as a single PFE in which plasty may be difficult or multiple PFEs regardless of impaired valve function.