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.

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.

The laparoscopic-endoscopic cooperative surgery for the colonic calcifying fibrous tumor: one case report.

Abstract The calcifying fibrous tumor (CFT) is classified as a benign fibrous lesion, and it is a rare pathologic entity. Local excision is the preferred therapeutic approach in treating CFTs. We present the first reported case in the literature of a CFT found in the colon, and this is the first time the way we did the laparoscopic-endoscopic cooperative surgery has been introduced.

[Doege-Potter syndrome. about one new case]. / Syndrome de Doege-Potter : à propos d'une nouvelle observation.

Syndrome Doege-Potter is a paraneoplastic syndrome in which hypoglycemia is the result of tumors producing insulin growth factor-like (IGF-II) it is most often solitary fibrous tumor of the pleura (TFSP). These are rare and may be discovered incidentally, during non-specific respiratory symptoms or during hypoglycemia. Hypoglycemia occurs in tumors of large volume and it disappears after surgery, which is the treatment of choice for a permanent cure in most cases. We present a case of Doege-Potter syndrome whose interest is to consider the TFSP as a cause of hypoglycemia in patients with pleural tumors.

Multifocal peritoneal calcifying fibrous tumour: incidental finding at cholecystectomy.

Calcifying fibrous tumour (CFT) is a benign tumour of elusive aetiology and a potential for local recurrence. Despite its peculiar histological characteristics it can still be confused with interrelated differential diagnosis like inflammatory myofibroblastic tumour (IMT) or solitary fibrous tumours. The clinical differential diagnosis is however much wider. To date seven cases of multiple peritoneal CFTs are on record. The authors present a case discovered incidentally during laparoscopic cholecystectomy, with no previous history and no radiological diagnosis achieved despite having undergone magnetic resonance cholangiopancreatography (MRCP) and normal routine perioperative investigation. The patient is disease-free 12 months after diagnosis. The case report is followed by a detailed literature review.

Multiple peritoneal calcifying fibrous tumors revealed by ischemic colitis.

We report the case of a 41-year-old man presenting with multiple calcifying fibrous tumor (CFT) revealed by ischemic colitis. Peritoneal nodular lesions involved omental, mesenteric, mesorectal, and mesocolic serosal surfaces. Histologically, nodules were composed of dense bundles of collagen, calcifications, and lymphoplasmacytic infiltrate. These findings were diagnostic of CFTs, a rare and often asymptomatic benign fibrous process. Multiple peritoneal CFTs are very uncommon and usually occur in women. Calcifying fibrous tumors are usually cured by surgical excision. The differential diagnosis and histogenesis of this entity are discussed.

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.

[Benign solitary fibrous pleural tumour and hypoglycaemia].

INTRODUCTION: Solitary fibrous tumours of the pleura are uncommon and discovered fortuitously or in patients with non-specific respiratory symptoms. When associated with hypoglycaemia, they are commonly referred to as Doege-Potter syndrome. CASE OUTLINE: A 68-year old woman presented with a large pleural mass. She had a long history of headache and decreased consciousness with one-year worsening dyspnoea and right-sided chest pain. The chest X-ray revealed an enormous opacity occupying almost the entire right hemithorax. Endocrine tests showed an extremely reduced glucose level and blood concentration of insulin, C-peptide, glucagon, growth hormone and catecholamines within normal range. After fine-needle aspiration, by histological and immunohistochemical analysis, a benign solitary fibrous pleural tumour was diagnosed. Although surgical resection of such a huge tumour with hypoglycaemia is usually curative, our patient declined surgery and opted for conservative treatment with intravenous glucose. CONCLUSION: Clinicians should be aware of this rare, but important cause of hypoglycaemia and exclude a fibrous pleural tumour in the assessment of their patients.

Solitary fibrous tumor associated with non-islet cell tumor hypoglycemia.

A 79-year-old man presented with abnormal fluttering movements of his extremities early in the morning. Fasting hypoglycemia was believed to be the cause of the movements. A computed tomographic scan showed a large mass in the left inferior hemithorax. Non-islet cell tumor hypoglycemia was suspected, and the mass was resected while the patient was under glucose supplementation therapy. The plasma glucose level became stable shortly after tumor excision. The resected tumor was diagnosed as a solitary fibrous tumor producing insulin-like growth factor II. In the follow-up examination approximately 2 years after the surgery, no recurrence of the tumor was observed, and the plasma glucose level was stable.

[Spontaneous hemothorax from a fibrous pleural tumour in an expectant father. Is the delivery room a dangerous place for men?]. / Spontan haemothorax fra en fibrøs pleural tumor hos en vordende far. Er fødestuer farlige for maend?

A 34-year-old male was admitted to our department with dyspnoea and severe pain in the left hemithorax. The symptoms had started suddenly three days before in the delivery room when his child was being born. An X-ray of the chest showed a tumour in the left side of the mediastinum and a large left pleural effusion. This was confirmed by CT, which revealed a round mass 11 cm in diameter. During the operation a benign solitary fibrous tumour was radically resected from the pleura and a large hemothorax was removed. This unusual presentation with severe bleeding from a pleural tumour was most probably initiated by the expectant father's excitement during his wife's labour.