Management of Pulmonary Artery Synovial Sarcoma: The 3-Step Surgical Approach.

Primary pulmonary artery sarcoma is an uncommon neoplasm. Given its clinical and radiographic resemblance to pulmonary embolism, initial diagnostic steps may be complicated, leading to delay in diagnosis. This report presents the case of a 52-year-old-woman who was admitted with pulmonary embolism. She underwent pulmonary embolectomy, and histopathologic examination revealed synovial sarcoma.

VASOPROLIFERATIVE TUMORS IN INTERMEDIATE UVEITIS.

PURPOSE: To describe patients with intermediate uveitis complicated by vasoproliferative tumors (VPTs). METHODS: Data were collected at seven Uveitis/Ocular Oncology centers on demographic, ophthalmic findings at baseline and at follow-up, and on imaging. The therapeutic intervention, final visual acuity, and duration of follow-up were recorded. RESULTS: A total of 36 eyes from 34 patients (12 men, 22 women; mean age 35.3 ± 14.2 years) were included in this study. Visual acuity at presentation ranged from 20/40 to counting fingers. At the time of VPT diagnosis, intermediate uveitis was active in all eyes. The mean VPT thickness was 3.06 ± 0.86 mm. Local treatment to the VPT was provide in 22 eyes (61.1%) and no local treatment to the VPT in 14 eyes (38.9%). After the VPT was detected, systemic or local treatment for the inflammation was initiated and on follow-up FAs 94.4% of the eyes showed resolution of the vascular leakage. During follow-up of 35.8 months, the 22 VPTs treated locally had a reduction in the tumor thickness to 1.25 mm, whereas the 14 VPTs untreated remained stable (final mean tumor thickness 2.65 mm). CONCLUSION: The presence of active intermediate uveitis accompanied by VPTs suggests the need for an aggressive uveitis treatment.

Reconstrução de couro cabeludo após ampla ressecção de angiossarcoma / Scalp reconstruction after wide resection of an angiosarcoma

Introdução: O couro cabeludo é uma área de difícil reconstrução devido à sua pouca elasticidade e por sobrepor-se a uma estrutura rígida e convexa. Existem diferentes técnicas cirúrgicas para reparação dos defeitos, que podem ser produto de diversas etiologias, como: traumas, deformidades e consequência de doenças, principalmente oncológicas, como é o caso do paciente apresentada neste trabalho. O angiossarcoma cutâneo é um tumor vascular maligno raro e extremamente agressivo, que afeta principalmente idosos. É caracterizado clinicamente pelo aparecimento de placas eritêmato-violáceas e de rápida evolução. O tratamento depende da extensão da doença. A maioria dos casos são tratados com ampla ressecção cirúrgica e reconstrução. O objetivo é relatar um caso de reconstrução de grande defeito do couro cabeludo depois de uma dissecção oncológica, realizado no Hospital Central do Exército (HCE) - RJ. Métodos: O caso foi tratado com enxerto autólogo e uso de matriz dérmica, em 2 tempos cirúrgicos, até a total cobertura da área lesionada. Resultados: Obteve-se resultado satisfatório após as cirurgias de enxertia de pele na área ressecada. Conclusão: O enxerto autólogo, juntamente a matriz dérmica mostrou-se uma opção viável na reconstrução do couro cabeludo.

Introduction: It is difficult to reconstruct the scalp due to its poor elasticity and presence of layers over a rigid convex structure. Different surgical techniques are used to repair defects that may develop due to several etiologies, such as trauma, deformities, and disease sequelae, especially cancer, as noted in the present case. Cutaneous angiosarcoma, a rare and extremely aggressive malignant vascular tumor that mainly develops in elderly individuals, is clinically characterized by the onset of rapidly evolving erythematous purple plaques. The treatment depends on disease extent. Most patients are treated with wide surgical resection and reconstruction. The objective is to report a case of reconstruction of a major scalp defect after an oncologic dissection performed at the Hospital Central do Exército in Rio de Janeiro. Methods: An autologous graft and dermal matrices were applied during two surgical periods till the damaged area was fully covered. Results: Satisfactory results were obtained after performing skin grafting surgery in the resected area. Conclusion: The autologous graft and dermal matrix proved to be a viable option for scalp reconstruction.

A Venous Malformation: An Unusual Primary Cardiac Tumor in Children.

This case report describes a primary cardiac tumor, classified as venous malformation, diagnosed in an asymptomatic child. The tumor was located in the left atrium near the mitral valve without affecting the mitral valve's functioning. Complete resection of the lesion was performed because of the risk of systemic embolism. The lesion consisted of fibrous tissue with multiple venous vascular channels. The patient did not have similar lesions in other locations. Vascular primary cardiac tumors are extremely rare. Hemangiomas and lymphangiomas have been described previously, but to our knowledge, this is the first primary cardiac tumor identified as a venous malformation.

Tumor de Masson intrahepático (hiperplasia endotelial papilar intravascular) / Intrahepatic Masson tumor (intravascular papillary endothelial hyperplasia)

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Hemangiopericitoma pélvico. Una localización infrecuente de tumor vascular / Pelvic hemangiopericytoma. An unusual location of a vascular tumor

Introducción: el hemangiopericitoma es una neoplasia infrecuente de estirpe vascular. Presentamos un caso clínico de interés dada su escasa frecuencia, tamaño y localización. Caso clínico: se trata de una mujer de 63 años que consultó por dolor abdominal. Se realizaron TAC, RMN abdominal y arteriografía que mostraban una gran masa pélvica que ocupaba el espacio de Douglas y desplazaba útero, vejiga y sigma y presentaba vascularización dependiente de la arteria mesentérica interior y ambas arterias hipogástricas. Se embolizó selectivamente la vascularización propia de la neoplasia y seguidamente se resecó la masa, sin presentarse complicaciones ni intra ni postoperatorias. La anatomía patológica confirmó el diagnóstico de hemangiopericitoma. La paciente sigue controles en consultas externas sin signos de recidiva hasta la fecha. Discusión: el hemangiopericitoma procede de las células del pericito por lo que puede presentarse en cualquier localización. La localización pélvica es excepcional. Puede presentarse como dolor abdominal inespecífico, dar síntomas de compresión de órganos vecinos y, ocasionalmente, asociarse a síndromes paraneoplásicos. El diagnóstico de sospecha es mediante TAC y angiografía aunque la confirmación es histológica tras analizar la pieza de resección. El tratamiento de elección es quirúrgico, precediéndose en algunos casos de embolización preoperatoria de la vascularización de la masa. No hay acuerdo sobre la quimio/radioterapia como tratamiento primario del hemangiopericitoma, aunque se ha descrito la radioterapia adyuvante para mejorar del control local y disminuir las recurrencias. El pronóstico es bueno si se consigue una resección completa, con supervivencias a 5 y 10 años entre el 70 y el 80% según las series (AU)

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Hombro doloroso: hemangioma en el músculo subescapular / Painful shoulder: hemangioma of the subscapularis muscle

Presentamos una tumoración vascular benigna, un hemangioma, situado en el músculo subescapular como causa de hombro doloroso en una mujer joven, caso poco frecuente en la literatura. Mujer de 30 años remitida a nuestra consulta por omalgia izquierda de 3 años de evolución, con radiografía y resonancia magnética cervical y de hombro izquierdo sin hallazgos significativos. Presenta dolor en la región escapular medial izquierda sin masa palpable. La resonancia magnética de la escápula informa de una tumoración vascular intramuscular, ovalada, bien definida, compatible con hemangioma subescapular. Tras la exéresis de la tumoración y el tratamiento rehabilitador posterior la paciente se encuentra asintomática. Los hemagiomas son tumores vasculares benignos que pueden manifestarse con dolor tras una actividad prolongada por la derivación del flujo sanguíneo que se produce desde el tejido circundante al hemangioma. Suelen tener buen pronóstico (AU)

We present a case of a benign vascular tumour, a haemangioma located in the subscapularis muscle as a cause of painful shoulder in a young woman. Such cases have rarely been reported in the literature. A 30-year-old woman was referred to our clinic for a 3-year history of shoulder pain and no significant findings on X-ray and magnetic resonance imaging of the cervical spine and left shoulder. The patient had pain in the left medial scapular area with no palpable mass. Magnetic resonance imaging of the scapula revealed an oval, well-defined, intramuscular vascular tumour compatible with haemangioma of the subscapularis muscle. After excision of the tumour and subsequent rehabilitation treatment, the patient is asymptomatic. Haemangiomas are benign vascular tumours that can cause pain due to swelling in the area of the tumour after prolonged activity. Prognosis is generally favourable (AU)

Managing Vascular Tumors-Open Approaches.

The most common vascular tumors encountered by the otolaryngologist are rare chromaffin cell tumors termed paragangliomas. Within the head and neck region, they commonly arise from the carotid body, vagus nerve (glomus vagale), and jugular vein (glomus jugulare). Other vascular head and neck tumors include sinonasal malignancies, because of proximity to or involvement of the pterygoid plexus as well as the rich vascularity of the sinonasal mucosa; juvenile nasopharyngeal angiofibroma, a vascular tumor of male adolescents; unusual vascular tumors such as hemangiopericytoma; and metastatic renal cell cancer, which has a proclivity for an unusually rich blood supply.

Core Biopsy of Vascular Neoplasms of the Breast: Pathologic Features, Imaging, and Clinical Findings.

Vascular lesions (VLs) of the breast present a diagnostic challenge on breast core biopsy (BCBx). We report on 27 VLs presenting on BCBx. The mean patient age was 60 years, and mean size was 7.5 mm (range, 1.6 to 16 mm). Presentation included palpable mass in 6 (22%), incidental in 6 (22%), and an imaging abnormality in 15 (56%) cases. Imaging impression included hematoma (24%), lymph node (10%), fat necrosis (10%), tortuous vessel (5%), and not provided in 52%. The lesions were classified on the basis of BCBx or BCBx and excision (available in 16 pts) as follows: 1 low-grade angiosarcoma, 8 angiolipomas, 6 capillary hemangiomas, 4 cavernous hemangiomas, 2 hemangiomas (not otherwise specified), 1 papillary endothelial hyperplasia, and 5 perilobular hemangiomas. The angiosarcoma was 9 mm, detected incidentally by magnetic resonance imaging, and showed dissection of stromal collagen, infiltration of glands, high cellularity, moderate cytologic atypia, scant mitotic activity, and Ki-67 reactivity of 10%. Among the 26 benign VLs, worrisome histologic features were noted in 14 on BCBx, including anastomosing vascular channels in 9, moderate cytologic atypia in 4, high cellularity in 2, Ki-67>10% in 2, mitotic activity in 1, and infiltration of glands in 1. Of the 12 VLs without worrisome features, the lesion extended to edge of core in 8, precluding complete evaluation. BCBx of VLs presents diagnostic challenges due to overlapping clinicopathologic and radiologic features with low-grade angiosarcoma. If completeness of removal is documented on BCBx, and cytoarchitectural changes are not worrisome, follow-up could be considered rather than excision. However, only 4 of these cases fulfilled those criteria.

Partial nephrectomy after selective embolization of tumor vessels in a hybrid operating room: A new approach of zero ischemia in renal surgery.

It is established that partial nephrectomy is the standard of care for tumors confined to the kidney. Achieving a partial nephrectomy without renal ischemia and limiting operative bleeding is the subject of numerous researches. Since 2010, hybrid operating rooms have been used to perform both interventional radiology and surgical procedures at the same place and time. We used this latest technology to treat 3 patients with localized kidney tumors. The tumors were of moderate complexity and all were treated after immediate hyperselective embolization by laparoscopic surgery without dissection and clamping of the renal pedicle. The embolization of tumor vessels could be performed using image-stitching software. After embolization, operative time was 50, 70 and 80 minutes and blood loss was less than 100 ml for each case. Postoperative control 3D arteriography confirmed the respect of the vascularization of the healthy renal parenchyma. No postoperative complications occurred. Combined approach including hyperselective embolization and partial nephrectomy in the same time in a dedicated operating room is a new approach of zero ischemia during partial nephrectomy which reduces the difficulty of the surgery, limits injury to the kidney and increases patient safety.

Espiradenoma ecrino vascular gigante / Giant Vascular Eccrine Spiradenoma

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Post-radiation atypical vascular proliferation on the head of a young woman: a diagnostic challenge.

With improved outcomes associated with radiotherapy (RT), post-irradiation tumors are increasingly seen in long-term cancer survivors. We report a case of a young woman who presented with a three-year history of a vascular lesion on the temple, previously irradiated for a childhood brain tumor. The history of radiation, the clinical appearance, and the biopsy findings of an atypical vascular proliferation in the dermis, were worrisome for a malignant vascular neoplasm and prompted surgical excision. However, further tissue analysis of the excised specimen confirmed a benign atypical vascular lesion (AVL) overlying a banal pilar cyst. Distinguishing post-radiation benign from malignant vascular lesions can be difficult because they share overlapping clinical and histopathologic features. Thus, any vascular lesion that occurs in a previously irradiated field should be excised completely with tumor-free margins and examined histologically.

Leiomyosarcoma of the great saphenous vein / Leiomiossarcoma de veia safena magna

A 56-year-old male patient presented with a complaint of two painful, hard, palpable nodules in the right lower limb. A Doppler ultrasound scan revealed the presence of nodules, likely to be neoplastic. Computed angiography showed two solid hypervascular nodules in the right great saphenous vein, fed by branches of the posterior tibial artery. Embolization of the nodules using surgical cyanoacrylate was performed, followed by an excisional biopsy. Anatomical pathology and immunohistochemical analysis identified the nodule as a high-grade leiomyosarcoma, characterized by ten mitotic figures per ten high-power fields, necrosis and cell pleomorphism. Immunohistochemical analysis results were positive for caldesmon and desmin labeling. A second surgical procedure was performed to enlarge the free margins.

Paciente do sexo masculino, 56 anos, com queixa de dois nódulos palpáveis, dolorosos e rígidos, em membro inferior direito. O eco-Doppler colorido evidenciou nódulos de provável natureza neoplásica na veia safena magna direita distal, com fluxo de baixa velocidade no seu interior. A angiotomografia evidenciou dois nódulos sólidos hipervascularizados no trajeto da veia safena magna direita, possuindo ramos nutridores provenientes da artéria tibial posterior. Para realizar a biópsia excisional dos nódulos, optou-se inicialmente pela embolização do tumor com cola cirúrgica de cianoacrilato, devido à sua alta vascularização. Os exames anatomopatológico e imuno-histoquímico evidenciaram leiomiossarcoma de alto grau, com dez mitoses por dez campos de grande aumento, necrose tumoral e pleomorfismo celular. A imuno-histoquímica demonstrou positividade para os marcadores Caldesmon (anticorpo hCD) e Desmina (anticorpo D33). O paciente realizou uma nova cirurgia para ampliação de margens comprometidas e está em acompanhamento clínico com a Oncologia.

Endoscopic transnasal interseptal approach for invasive clival tumors: development of an approach method regarding maximal preservation of the nasal anatomy.

Asian people frequently show small noses, narrow nasal apertures, and congestive mucosa on the turbinates and septum. To reduce the risk of nasal morbidity with increased radicality for skull base tumors in these patients, we developed endoscopic transnasal interseptal approach (ETISA) in transsphenoidal surgery for invasive clival tumors (ICTs). Indication for ETISA is radical resection for tumors occupying deep ventral skull base regions, confined posterior to the level of the middle turbinates. After removing ethmoidal air cells, the middle turbinates are laterally deflected. A linear incision is made vertically on each side of the septal mucosa, which is separated from the bony septum as far as the sphenoid rostrum. The blades of an adjustable speculum are inserted submucosally, and the bony septum is temporarily displaced. The surgical pathway is widely maintained from the entrance to the deep surgical field without sacrificing the nasal mucosa and turbinates. Thirty-two consecutive patients with clival tumors (18 chordomas, 11 chondrosarcomas, 3 others) were treated. Bilateral middle turbinectomy was routinely performed in the initial 3 patients, but in only 4 of the remaining 29 (3 unilaterally, 1 bilaterally). Tumor was sufficiently resected in 29 patients (90.6%; gross total removal n=25, subtotal resection n=4). As complications, 6 patients showed mild and transient worsening of cranial nerve symptoms. Nasal complications arose in 3 patients, persisting >3 months in 2 (5.7%). This approach allows fine bimanual handling and swift delivery of surgical equipment while reasonably preserving the nasal anatomy, which is useful in endoscopic transsphenoidal surgery for ICT.

Thirteen-year follow-up of parasellar intravascular papillary endothelial hyperplasia successfully treated by surgical excision: case report.

Intracranial intravascular papillary endothelial hyperplasia (IPEH) is very rare, and to our knowledge long-term follow-up results have not been previously published. An 11-year-old boy presented with a 6-month history of progressive visual impairment in the right eye. Magnetic resonance imaging revealed a well-enhanced, large parasellar mass involving the cavernous sinus, right frontal skull base, and ethmoid and sphenoid sinuses. Frontotemporal craniotomy and subtotal resection were performed, and the diagnosis of IPEH was confirmed. The mass increased in size during the following 3 months. A second operation was performed via frontotemporal craniotomy combined with a transsphenoidal approach, and gross-total resection of the tumor was achieved. Adjuvant radiotherapy (5040 cGy) and chemotherapy with interferon were administered. The patient's visual symptoms improved, and there was no recurrence during a 13-year follow-up period. The results of this case indicate that intracranial IPEH can recur with subtotal resection; however, optimal resection with multimodal adjuvant treatment can control the disease for many years, if not permanently.

Diagnosis and surgical treatment of cavernous sinus angioleiomyoma: a report of four cases.

OBJECTIVE: The aims of the study were to retrospectively evaluate the imaging characteristics of cavernous sinus angioleiomyoma and explore appropriate surgical approaches. METHODS: A total of four cases admitted to our hospital for repeated episodes of headache were enrolled. The common clinical manifestations included decreased vision, diplopia, blepharoptosis and facial numbness. The first three patients were misdiagnosed with cavernous sinus hemangioma, meningioma and chordoma, respectively. The fourth case was suspected to be angioleiomyoma. Imaging examination showed an oval-shaped tumor occupying the cavernous sinus. The tumor was hypointense on T1-weighted magnetic resonance imaging and extremely hyperintense on T2-weighted scan, which was similar to the cerebrospinal fluid, and was progressively enhanced from one side after gadolinium injection, pushing the internal carotid artery toward the inner side. RESULTS: Surgical resection was performed via an epidural approach for these four cases. Clinical symptoms were improved after surgery and no recurrence was observed during follow-up visits (average, 47.5 months). CONCLUSIONS: The initial symptom of cavernous sinus angioleiomyoma was repeated headache. The tumor seems extremely hyperintense on a T2-weighted image and expansive growth is pushing away the internal carotid artery rather than encasing it. It was progressively enhanced from one side after gadolinium injection. Surgical treatment based on an epidural approach had an excellent outcome in tumor resection and nerve protection.