Síndrome de Mondor / Mondor's syndrome

No disponible

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.

Defecto fibroso cortical, por favor no tocar / Fibrous cortical defect, please do not touch it

El defecto fibroso cortical es una entidad común en la población infantil y su morfología radiológica típica permite el diagnóstico; dado su carácter involutivo no requiere tratamiento. Sin embargo, el desconocimiento de esta patología por parte de los pediatras conlleva un aumento de la demanda de exámenes complementarios y es generador de situaciones de estrés en pacientes y familiares. Se presenta el caso de un niño con defecto fibroso cortical recientemente diagnosticado en nuestro centro (AU)

The fibrous cortical defect is a common entity in children and the typical radiological image enables diagnosis. As these lesions are self-limited, no treatment is required. However, this lesion is not well known by pediatricians, which leads to an increased use of complementary tests and generates stress on patients and families. We present the case of a child with fibrous cortical defect recently diagnosed in our center (AU)

Testicular fibrous pseudotumour as a rare intrascrotal mass / El seudotumor fibroso testicular como masa rara interescrotal

Introduction. Paratesticular fibrous pseudotumor (PFP) is a rare benign tumor that is not a neoplasm but a reactive fibrous inflammatory hyperplasia. Paratesticular fibrous pseudotumor occurs from intrascrotal tissues, such as tunica vaginalis, epididymis, or spermatic cord. Definitive diagnosis requires pathological examination. Radical orchiectomy should be avoided when possible, and local excision should be performed due to the lack of obvious evidence of potential malignancy. Case report. A 61 years old patient was referred to our clinic with complaints of right scrotal mass. Doppler ultrasonography is observed for the right epididymis in the neighborhood of 30 × 26 × 21 mm hypoechoic mass. Tumor markers were negative. Frozen came as negative. Testicular mass was excised with testis preventive approach. Pathology report revealed that paratesticular fibrous pseudotumor. Conclusion. According to this article frozen section should be done in paratesticular masses to avoid unnecessary radical orchiectomy (AU)

Introducción. El seudotumor fibroso paratesticular es un tumor benigno raro que no es una neoplasia sino una hiperplasia o proceso reactivo inflamatorio no tumoral. El seudotumor fibroso paratesticular aparece en el tejido intraescrotal, como la túnica vaginal, epidídimo, o el cordón espermático. El diagnóstico definitivo requiere un examen patológico. Debe evitarse la orquiectomía radical en la medida de lo posible. Asimismo, deberían realizarse escisiones locales en caso de no encontrar una evidencia clara de su potencial malignidad. Informe del caso. Un paciente de 61 años dirigido a nuestra clínica con quejas de una masa en el escroto derecho. Se observa una masa hipoecoica de 30 × 26 × 21 mm en las proximidades del epidídimo derecho durante el examen con ecografía doppler. Los marcadores tumorales fueron negativos. Se realizó biopsia de la masa como procedimiento preventivo testicular. El informe patológico confirmó que se trataba de un seudotumor fibroso paratesticular. Conclusión. De acuerdo con este artículo, la biopsia del tejido congelado debería realizarse en masas paratesticulares para evitar orquiectomías radicales innecesarias (AU)

Tumor fibroso solitario de cavidad oral. Confirmación histológica final ante la sospecha de lipoma facial / Solitary fibrous tumor of the oral cavity. Final histologic confirmation after suspected facial lipoma

No disponible

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.

Un nuevo caso de histiocitoma fibroso maligno dependiente de cápsula renal / A new case of malignant fibrous histiocytoma arising from the renal capsule

No disponible

No disponible

Pseudotumor fibroso paratesticular / Paratesticular fibrous pseudotumor

Objetivos: Los procesos proliferativos benignos testiculares se conocen con el nombre de pseudotumors, al no ser considerados en la actualidad verdaderas neoplasias. El Pseudotumor Fibroso Paratesticular (PFP) es una entidad sobre las que existe gran confusión en su definición y etiopatogenia. Se trata de un proceso que confunde al clínico al aparecer simulando neoplasias. La presente comunicación puede ayudar a conocer mejor este proceso no tumoral y evitar confusiones diagnósticas. Métodos/Resultados: Revisamos nuestra serie de PFP (tres casos) sobre esta rara entidad, por lo que dada su rareza puede considerarse significativa. Analizamos su clínica, su comportamiento antes los estudios diagnósticos por imagen, hallazgos operatorios y evolución. Conclusiones: El PFP es una rara entidad que puede confundir al clínico al ser diagnosticado como una neoplasia, y conllevar tratamientos agresivos (orquiectomía) que de realizar un exacto diagnóstico podrían obviarse. El análisis de una serie de tres casos, con sus aportaciones, y la revisión de la literatura efectuada pueden ayudar en la practica clínica al urólogo a reconocer esta enfermedad (AU)

Objective: Benign testicular proliferative processes are known by the name of pseudotumors, because currently they are not considered real neoplasias. The paratesticular fibrous pseudotumor (PFP) is a rare entity, the definition and etiopathogenesis of which is under great confusion. It is a process that misleads the clinical because they simulate neoplasias. This paper aims to help a better knowledge of this non-tumoral process and to avoid diagnostic confusions. Methods/Results: We review our series of PFP (three cases), which can be considered significant due to its oddity. We analyze the clinical picture, its behavior, imaging diagnostic tests, operative findings and outcomes. Conclusions: PFP is a rare entity which may misleads the clinical because it may be diagnosed as a neoplasia and lead to aggressive treatment (orchiectomy) which in case of a proper diagnosis could be avoided. The analysis of our series of three cases, with their contribution, and a literature review may help the clinical practice of urologists by recognizing this disease (AU)

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.

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.