Intestinal perforation as a form of presentation of a small bowel calcifying fibrous tumor.

Calcifying fibrous tumor (CFT) is a rare, benign, mesenchymal tumor. It has a slight female predominance, and it can appear in any range of age. It can be in the extremities, neck, and gastrointestinal tract, but it has also been described in other locations. Even though it is a benign lesion, recurrence has been described in some cases in the literature. A free-margin surgical resection is the recommended treatment. We present a 56 -year-old woman who underwent surgery for an intestinal obstruction associated with middle jejunum perforation. Histopathological study described the presence of a calcifying fibrous tumor. Spindle cells were positive for CD34, Factor XIIIa and vimentin. To our knowledge, this is the first case of intestinal perforation secondary to a calcifying fibrous tumor described in the literature.

Immunoglobulin G4-related paratesticular fibrous pseudotumor and retroperitoneal fibrosis: a case report.

A 46-year-old man with a past history of retroperitoneal fibrosis was admitted with an enlarged, hard right testis. The paratesticular lesion showed heterogeneous hypoechogenicity on ultrasonography, low signal intensity on T1- and T2-weighted magnetic resonance imaging (MRI), and lack of diffusion restriction on diffusion-weighted MRI. Following steroid treatment, the paratesticular mass was decreased in size on follow-up computed tomography. The radiologic and clinical features are recognized as a manifestation of immunoglobulin G4-related sclerosing disease involving the paratesticular region and retroperitoneum.

Acquired von Willebrand syndrome with a type 2B phenotype: diagnostic and therapeutic dilemmas.

In this report, we provide evidence of an acquired von Willebrand syndrome (AVWS) with a type 2B phenotype rather than the expected type 1 or 2A. The patient was referred prior to surgical removal of a fibrous mass within the maxillary sinus. His first bleeding 7 years earlier following a retinal tear had been complicated by monocular blindness. Several mucocutanous bleedings followed. Hematological investigations revealed von Willebrand factor (VWF):Ag 91 IU/ml, factor VIII 86 IU/ml, VWF:RCo 34 IU/ml and profound thrombocytopenia with platelet clumping. VWF multimer analysis showed a loss of high-molecular-weight multimers and his plasma aggregated normal platelets under low ristocetin concentration, consistent with type 2B von Willebrand disease (VWD). Sequencing of VWF exon 28 and of the platelet GP1BA gene to investigate the possibility of platelet-type VWD failed to reveal mutations. Serum protein electrophoresis showed a monoclonal IgG protein and led to the diagnosis of monoclonal gammopathy of unknown significance (MGUS), raising suspicion of an AVWS. Over 2 years, he experienced severe gingival bleedings and traumatic intracerebral hemorrhage. Following debridement of the sinus mass, the patient required 20 units of packed red blood cells, despite high-dose Humate-P, continuous Amicar and twice-daily platelet transfusions. Bleeding finally ceased following infusion of activated factor VIIa. A history of prior uncomplicated vasectomy and tendon laceration, no family history of bleeding, the inability to identify a causative mutation in either exon 28 VWF or platelet GP1BA and the MGUS led to diagnosis of AVWS with a type 2B phenotype. This case highlights the difficulties in assigning a diagnosis and the management of bleeding in a patient with an atypical presentation of AVWS.

Large solitary fibrous tumour of the retroperitoneum: a case report and review of the literature.

INTRODUCTION: This report describes an unusual case of a large solitary fibrous tumour (SFT) arising in the retroperitoneum. CASE PRESENTATION: A 53-year-old woman presented at the Emergency Department with urinary retention and lumbar pain. The urological examination was negative, whereas a presacral retroperitoneal mass was disclosed on ultrasound. The laboratory studies, including tumour markers, were negative. On laparotomy, it was not possible to remove the mass completely due to the difficulty of dissecting it free of the sacrum. Grossly, the fragment had a yellowish-white surface, with areas of necrosis and haemorrhage. On immunohistochemistry, tumour cells were positive for CD34, CD99 and Bcl-2 and negative for CD45, synaptophysin, chromogranin, S100, neuron-specific enolase, CK AE1-AE3, CK7, Wilms' tumour 1, smooth muscle actin, factor VIII, myogenin, epithelial membrane antigen, thyroid transcription factor-1 and CD117, leading to a diagnosis of SFT. Molecular investigation ruled out synovial sarcoma. CONCLUSION: Although SFT usually has a favourable prognosis, close follow-up is recommended due to the limited information on its long-term behaviour.

Secondary hypertrophic osteoarthropathy associated with solitary fibrous tumor of the lung.

Secondary hypertrophic osteoarthropathy, synonymous with the terms "pulmonary hypertrophic osteoarthropathy" and "pachydermoperiostosis acquisita," is a syndrome characterized by finger clubbing, hypertrophic skin changes, and periosteal bone changes that can occur in patients with underlying disease of the cardiovascular and pulmonary systems. We report the case of a 56-year-old man with a solitary fibrous tumor of the lung causing symptomatic hypoglycemia and secondary hypertrophic osteoarthropathy. The patient was seen in the dermatology clinic because of his skin changes. Diagnostic work-up included imaging that revealed a large mass in the left lower lobe of his lung later identified as a solitary fibrous tumor. The patient underwent pneumonectomy and at 1-month follow-up had returned to a euglycemic state with resolution of his skin findings. Dermatologists may play a key role in early diagnosis of tumors of the lung and other extrathoracic sites, such as solitary fibrous tumors, as well as many endocrine disorders, through the recognition of the cutaneous and soft tissue manifestations.

The evolution of a pleural nodule into a giant fibrous tumor associated with hypoglycemic coma.

We hereby present the exceptional case of hypoglycemic coma associated with a giant benign localized fibrous tumor of the pleura (LFTP). A 79-year-old woman was found to have a small peripheral nodular lesion in the right hemithorax 7 years ago. A fine needle aspiration biopsy provided a diagnosis of LFTP, but the patient refused surgery. Six years later hypoglycemic coma and respiratory insufficiency appeared in association with a giant mass completely filling the right chest and shifting the mediastinum contralaterally. The tumor was completely resected, and complete relief of the hypoglycemic and respiratory symptoms was achieved. The patient is in good metabolic and respiratory conditions and disease-free 14 months after the operation.

Ruptured Infantile Myofibroma of the Head Presenting in a Neonate: Case Report and Review of the Literature.

BACKGROUND: Infantile myofibroma/myofibromatosis (IM/M) is a myofibroblastic proliferative disorder often seen in infants and children. IM/M can result in congenital tumors of the head and neck and may occasionally present to the neurosurgeon. CASE DESCRIPTION: We report a case of a solitary ruptured myofibroma of the head in a newborn patient. The lesion was initially suggestive of encephalocele. We describe the presentation and management of this patient, including relevant imaging, histopathologic evaluation, and surgical technique. We subsequently review the literature of IM/M of the head and neck, highlighting the 3 forms of the condition, each requiring a distinct management strategy. CONCLUSIONS: Although this tumor rarely presents to the neurosurgeon, it may do so in the process of ruling out other more dangerous conditions. It is therefore important to consider this diagnosis in masses that occur in the head and neck of newborns.

Pleural malignant solitary fibrous tumor with sarcomatous overgrowth showing PDGFRbeta mutation.

Pleural malignant solitary fibrous tumors (SFTs) are uncommon, and little is known about their histogenesis and molecular features. We report a case of pleural SFT with sarcomatous overgrowth that showed expression for PDGFRbeta and a missense mutation on exon 18 of the PDGFRbeta gene. The involvement of the PDGFRbeta gene in SFT is compatible with a pericytic derivation, also supporting a possible role of this tyrosine kinase in malignant transformation and in the adoption of novel molecular therapies.

Intermittent respiratory obstruction secondary to a solitary fibrous tumour.

There have been fourteen cases of solitary fibrous tumour reported as originating from the paranasal sinuses. Here we report a case of solitary fibrous tumour that involved the right nasal cavity with extension into the oropharynx causing stertor and intermittent respiratory obtruction. Histopathology examination revealed the tumuor cells expressed CD34 turnout marker.

Fibrosing inflammatory pseudotumors of the central skull base.

The most important step in the differential diagnosis of mass lesions of the central skull base is to rule out malignant neoplasms. However, nonneoplastic lesions, such as infections or nonspecific inflammatory lesions of the skull base, can mimic malignant processes. In this study, the authors analyzed seven cases of nonneoplastic noninfectious mass-forming lesions involving the central skull base. In most cases, malignant processes were suspected at the initial phase of diagnostic work-up, but subsequent histologic examinations revealed that these lesions consisted of inflammatory cells and fibrosis without neoplastic cells. Common manifestations were pain and other neurological symptoms related to the involved anatomical sites. A variety of neurological dysfunctions of the cranial nerves not including the olfactory and spinal accessory nerves were observed. No patient developed separate lesions outside the head and neck region. After the pathologic diagnosis, most of the patients were treated with oral steroid therapy, with initial doses of prednisolone, 60 to 100 mg/d. It was difficult to relate responsiveness to steroid therapy with the histologic degree of sclerosis, fibrosis, or chronicity of the disease in these cases. Otolaryngologists should be aware of this disease when making treatment decisions for their patients with skull base lesions.

Fibrous pleural tumor with hypoglycemia: case study.

Many neoplastic tumors exhibit paraneoplastic syndromes manifested by endocrinopathy. This is particularly true of intrathoracic tumors such as lung cancers, thymomas, carcinoid tumors and mediastinal germ cell neoplasm. Fibrous tumors of the pleura are rare intrathoracic tumors, which are usually benign and often grow to huge size. A subset of these neoplasms present with the syndrome of hypoglycemia. Although first reported more than 70 years ago, the diagnosis is rarely considered when a patient presents with syncope and hypoglycemia. This article reports a patient who presented with a large pleural mass and a hypoglycemic syndrome. (The disease was surgically cured.) The probable mechanism of hypoglycemia is discussed.

An unusual presentation of solitary fibrous tumour of the pleura: right atrium and inferior vena cava compression.

Solitary fibrous tumours of the pleura are rare and usually present with unpredictable clinical signs. We report a 58-year-old patient presenting with signs of cardiac decompensation and external compression of the right atrium and inferior vena cava by a voluminous solitary fibrous tumour arising from the diaphragmatic pleura. Marked clinical improvement was obtained after excision of the mass with complete disappearance of the cardiac signs.

Doege-Potter syndrome: hypoglycemia associated with malignant solitary fibrous tumor.

We report a case of recurrent solitary fibrous tumor with associated hypoglycemia, the "Doege-Potter syndrome." These are rare tumors with a 12-13% rate of malignancy. The syndrome of hypoglycemia is seen in less than 5% of the cases, and the associated tumors are large with a high mitotic rate. The cause of hypoglycemia is related to insulin-like growth factors produced by these tumors. Resection in many cases is curable with resolution of the hypoglycemia. We discuss the different signs and symptoms and literature evidence of treatment options and follow-up.

Arnold's neuralgia caused by solitary fibrous tumor. Case report.

Solitary fibrous tumor (SFT) is a mesenchymal tumor which has been identified in a wide variety of localizations, including soft tissues, peritoneum, retroperitoneum, mediastinum, upper respiratory tract, nasopharyngeal sinuses, periosteum and extremities, orbit, major body cavities, intraspinal and intracranial localizations. The authors describe a case of SFT found in the neck of a young patient suffering from Arnold's neuralgia. After surgery, diagnosis of SFT was based on characteristic histopathological findings, especially on immunohistochemical positive staining for CD34 antigen. It has been described also the characteristic patterns making this diagnose sure, focusing the point that cure is possible with complete excision of the lesion.

Solitary fibrous tumor of the parapharyngeal space.

Solitary fibrous tumors are rare tumors originating from the mesenchymal tissue. In most cases they arise from the subpleural tissue but have also been described in other locations in the body. Most such tumors in the head and neck region occur in the orbit. Although malignant tumors can occur, benign tumors are far more common. The therapy is surgical excision. For a conclusive diagnosis, a typical morphology and immunohistochemistry are required. The tumor is strongly positive for CD34 and vimentin. This report describes the clinical appearance and treatment of a solitary fibrous tumor of the parapharyngeal space in a female patient. The symptoms were similar to those in a peritonsillar abscess, one of the more common ear-nose-throat dysfunctions. Fewer than 10 cases of a solitary fibrous tumor of the parapharyngeal space have been described in the literature.