Nasopharyngeal angiofibroma: staging and management - a review of the case series report

Juvenile angiofibroma is a rare, highly vascular, histologically benign but locally invasive tumor, affecting predominantly male adolescents. Here we present a case series of 45 patients of nasopharyngeal angiofibroma, their clinical presentation, staging and the various surgical approaches for their excision.     

Juvenile nasopharyngeal angiofibroma: a single institution study

BACKGROUND: Juvenile nasopharyngeal angiofibroma (JNA) is a rare tumor of adolescent males and there is a paucity of Indian studies on this subject. AIMS: To present the experience of management of JNA at a single institution. SETTING AND DESIGN: This is a retrospective observational study of patients with JNA who presented at the Tata Memorial Hospital between May 1988 and August 2001. MATERIALS AND METHODS: Thirty-two patients with JNA were treated in the study period. Since the time period was prolonged and diagnostic and therapeutic protocols had undergone many changes, the patients were divided into two groups, namely 1988-1996 and 1997-2001. The age distribution, disease patterns, management approaches and treatment outcomes of patients in the two groups were recorded. Statistical analyses were done using students 't' test and test for proportion. RESULTS: The mean age at presentation was 16 years and more than 90% of the patients had Stage III or IV disease. Preoperative embolization was carried out in 19 patients. The surgical approaches used were median maxillectomy, infratemporal fossa, transpalatal, maxillary swing and craniofacial approach. The recurrence rate, complete resection rate and cure rates were 12.5%, 41% and 63% respectively. CONCLUSION: Surgery is the mainstay of treatment of JNA. Preoperative embolization and newer surgical approaches result in less blood loss and complete resection. Aggressive re-resection should be done for resectable recurrences reserving radiotherapy for unresectable, recurrent/ residual disease.     

Juvenile nasopharyngeal angiofibroma: current treatment modalities and future considerations

Juvenile angiofibroma (JNA) is a relatively uncommon, highly vascular and benign tumor that presents most commonly in adolescent males. Symptoms may persist from months to years and often times, these tumors are asymptomatic until they increase and encroach on critical structures. Because of technological advances both in surgery and radiology, management of JNA patients has been refined. With the advent of more sophisticated capabilities such as CT, MRI, intensity-modulated radiation therapy (IMRT), stereotactic guidance systems as well as advanced embolization techniques, these tumors can be diagnosed and managed more effectively. Patients with juvenile angiofibroma (JNA) are typically silent for years and often present with epistaxis, nasal obstruction, facial numbness, rhinorrhea, ear popping, sinusitis, cheek swelling, visual changes and headaches. In addition to these symptoms, up to one-third of patients with this condition may present with proptosis or other orbital involvement, which are late symptoms and findings. Most physicians agree that surgery is the primary treatment modality for the early-stage disease process. However, controversy arises regarding the best treatment when a patient presents with more locally advanced disease involving widespread cranial-based extension or intracranial involvement which may necessitate a combination of treatment modalities including surgery and postoperative radiation. With the advancement of endoscopic surgery, there have been a number of cases reporting the value of its use. The purpose of this review, however, will address not only endoscopic alternatives, but will discuss other treatment options as reported in the literature. Robotic surgery of the skull base for JNA is something to expect for the future. Finally, with the advent of IMRT and an image-guided robotic radiotherapy delivery system, some researchers speculate that this will result in less objections for radiation in general and certainly less reservations for the use radiotherapy in certain circumstances, i.e. patient refusal of surgery or extensive non-resectable or recurrent JNA tumors.     

Juvenile nasopharyngeal angiofibroma—our experience at a referral hospital

Nasopharyngeal angiofibroma is a rare, highly vascular locally invasive tumor with a strong tendency to bleed. It usually occurs in young adolescent males. This is a retrospective study of 53 patients with nasopharyngeal angiofibroma. All the cases were treated surgically by different conventional approaches depending upon the extent of the mass. Transpalatal approach was used in 32 cases, lateral rhinotomy in 13 cases and a combination of transpalatine + lateral rhinotomy + caldwel-luc in 8 cases. There is no recurrence in 49 cases till date. However, recurrence was noted in four cases within six months. Three out of four patients with recurrence had to undergo repeat surgery for the removal of angiofibroma within six months while the remaining one was referred to radiotherapy as the mass was invading the cavernous sinus. The various clinical presentation and treatment modalities are discussed.     

Sarcoidosis and lymphoma: case series and literature review

This is a case series and literature review of patients with various types of hematologic disorders and sarcoidosis. The patients were treated in the hematologic day care unit at Shaare Zedek medical center and at the hematology department of Chaim Sheba Medical Center hospital from 1990 until 2008. We report eight cases from two centers where both diseases were diagnosed at different time points. We have shown that there might be coincidence of two rare conditions and emphasize the importance of tissues sampling of lesions suspected to be a relapse of a former biopsy-proven disease, even in the presence of positive PET results.     

Aggressive angiomyxoma: A case series and literature review

Background

Aggressive angiomyxoma was identified as a distinct clinicopathologic entity in 1983 and since then fewer than 250 cases of these rare tumors have been reported in world literature. These tumors usually arise in the pelvis and perineal regions, most often in women of the reproductive age group; however a few cases of its occurrence outside the pelvis have also been reported.

Patients and methods

We report a series of 7 women treated in our institute in the last 8 years. Relevant literature on aggressive angiomyxoma was looked at and various management options reviewed.

Conclusion

Aggressive angiomyxomas are locally aggressive, notorious for local recurrence and extremely rare to metastasize. While surgery remains the mainstay of treatment, there has been a definite shift towards less radical forms of excision, over the years. Various adjuvant treatment modalities have also been tried to reduce tumor recurrence.     

Preoperative embolization of a large vaginal leiomyoma: Report of a case and review of the literature

Leiomyoma of the vagina is a very rare tumour of the lower urogenital tract. These slow‐growing masses may be asymptomatic or present with pain, dyspareunia or urinary symptoms. Rarely, these tumours may present with life‐threatening haemorrhage. These hypervascular tumours are treated by surgical excision. Preoperative embolization therefore may aid in devascularization of these tumours before surgical excision. We present the MRI features of a case of vaginal leiomyoma, which was managed by preoperative embolization and was then excised in toto. To the best of our knowledge, this is the first report where preoperative embolization was performed before excision of a vaginal leiomyoma with minimal peroperative blood loss.     

Atypical angiofibroma of larynx — a case report

Primary extra nasopharyngeal angiofibroma of larynx is a very rare tumour. We here by present a case of angiofibroma of larynx affecting the anterior commissure & subglottic region in larynx-a rare site of involvement.     

Juvenile psammomatoid ossifying fibroma: a review

In WHO classification of odontogenic tumors (2005), juvenile ossifying fibroma (JOF) is divided into juvenile psammomatoid ossifying fibroma (JPOF) and juvenile trabecular ossifying fibroma (JTOF). JPOF has been distinguished because of its location, clinical behavior, and age of occurrence. It is generally seen in younger age group and the most common site is paranasal sinuses, orbits, and fronto-ethmoidal complex. Radiologically, the internal structure can be radiolucent, mixed, or radiopaque, depending on the degree of calcification and extent of the cystic changes. Histologically, it is characterized by a densely cellular fibrous stroma interspersed with numerous psammoma bodies. The treatment is "en bloc" surgical excision while in case of intracranial extension, tumor removal may need a combination of neurosurgical and transfacial approach. Malignant transformation and metastasis has not been reported but recurrence is common. The purpose of this narrative review article is to discuss the various aspects of JPOF reported in the English medical literature.     

Importance of pre-operative embolization in the surgery of nasopharyngeal angiofibroma

Juvenile Nasopharyngeal Angiofibroma are rare vasoformative¹ neoplasms of the Nasopharynx. Due to innocuous nature of their presenting symptoms, the tumour frequently has extranasopharyngeal extensions at the time of initial diagnosis. The advances in radiographic CT techniques² and pre-operative embolization³ have reduced the mortality and morbidity to a great extent. We analysed nine cases of angiofibroma with and without embolization and concluded that preoperative embolization reduces intraoperative blood loss considerably giving excellent surgical clearance of the tumour mass with minimal risk of recurrence.     

Management principles of head and neck cancers during pregnancy: a review and case series

Head and neck cancers during pregnancy are rising in incidence. Clinicians need to be aware of the principles of managing these cases. This paper is a review of the literature of head and neck cancers during pregnancy with a short case series of six non-thyroid pregnancy-associated cancers treated by a head and neck oncology surgeon. The age range is 20-34 years. Tumour types include carcinoma of the tongue, nasopharynx, maxillary sinus, parotid gland, subglottic region, and the neck. Five patients were treated surgically and four were treated with either chemotherapy or radiotherapy. Both foetal and maternal death occurred in two cases. The range of follow-up is 15 months to 14 years. Managing head and neck cancers during pregnancy requires the addition of a pregnancy-related layer of understanding to the armoury of existing specialist knowledge, encompassing a triad of effects, (i) the aetiological effect of pregnancy on cancer, (ii) the direct and indirect effects of cancer on pregnancy, and (iii) the effect of diagnostic and treatment modalities on pregnancy. Consideration must also be given to the ethical dilemmas of decision making.     

Juvenile nasopharyngeal angiofibromas: A study of recurrence pattern and role of pre-Operative embolization - ‘a decade’S experience’

This study is a retrospective analysis of 30 consecutive cases of Juvenile Nasopharyngeal Angiofibroma (JXA) operated at. Department of Head and Neck Surgery, Kidwai Memorial Institute of Oncology Bangalore, India: la tertiary referral centre) after prior emohilization by an interventional neuro-radiologisl (1996-2002). This study discusses critically the planning of surgical approach, based on anatomico-radiological factors and highlights the efficacy of preoperalive embolization in expediting total re moral of the tumor in 25 out of JO cases with advanced stage JNA. Objectives: To analyze the utility of pre-operatire embolisation in surgical extirpation of large JNAs; planning of the surgical approaches based on CT topography of the tumor; to study the various complications of embolisation and surgery associated with JXA & lastly to evaluate the puttern and location of recurrent tumor thus correlating with the original topography. Setting: Tertiary care cancer referral centre. Patients: Patients ranged in age from ’)- 24 years. all being males. Interventions: Majority of them were accessed by transfacial surgical approach(26). and in the recent past via midfacial degloving(4) within 4H hours of angioembolisation. Results: Complete removal of the tumor was achieved in 25 out of 30 cases with advanced stage JNA. Post surgical CT scans revealed tumor residua in 5 individuals, where the tumor was documented in - the temporal fossa 12), para-cavernous sinus region (I), cavernous sinus! I) and pterygo palatine fossa (I). Only the lesion in pterygopalaline fossa was successfully re-i>xcised & this alongwith the recurrence at para-cavernous & cavernous sinus & another were treated with radiotherapy; the 2 cases in the temporal fossa are under observation. The average blood loss during the procedure was 546.60 ml. Conclusions: Today, advances in radiologie imaging-complemented by interventional neuro-radiological expertise in angio-embolisation have expedited complète excision with minimal morbidity and acceptable recurrence rate. This study has justified pre-operative embolisation and M currently the standard of care for advanced JXA.     

Management of bevacizumab-associated bowel perforation: a case series and review of the literature.

BACKGROUND: This study examined the various approaches to the management of perforation and the associated outcomes in patients with bevacizumab-associated bowel perforation at a tertiary cancer center. PATIENTS AND METHODS: Our institutional pharmacy database was searched to identify all patients who had received bevacizumab over a 2-year period (January 2004 to October 2006). Medical records of these patients were examined for reports of confirmed bowel perforation or fistula, associated clinicopathological factors, treatment, and outcomes. RESULTS: We identified 1442 patients who had been treated with bevacizumab over the study period with perforation occurring in 24 (1.7%). The breakdown of these 24 patients by disease site was as follows: ovarian (3 of 50, 6%), gastroesophageal (2 of 38, 5.3%), pancreatic (7 of 141, 5%), unknown primary (1 of 60, 1.7%), lung (1 of 67, 1.5%), colorectal (6 of 478, 1.3%), and renal cell (4 of 269, 1.5%). The majority of patients (n = 19, 79%) were initially managed nonoperatively. Only five (21%) patients ultimately underwent surgical exploration, with a subsequent anastomotic leak developing in one patient. The overall 30-day mortality rate was 12.5%. CONCLUSIONS: Bevacizumab-associated bowel perforation occurs in patients with various malignancies, with an incidence of 1.7%. Nonoperative treatment is a viable approach to management in selected patients.     

三羟甲基-丙烯酸微球栓塞鼻咽和鼻窦纤维血管瘤疗效的观察

目的：评价一种新型栓塞材料（三羟甲基-丙烯酸微球）在鼻咽和鼻窦纤维血管瘤介入治疗中的有效性。方法：经股动脉穿刺,导管进入一侧或两侧颈外动脉供血血管,以直径100～500μm三羟甲基-丙烯酸微球栓塞肿瘤供血动脉。栓塞后1～3d行外科手术切除。结果：栓塞后,11例接受手术完整切除,术中见肿瘤均有缩小,部分肿瘤已发生坏死,术中出血150～900mL（平均380mL）。均经病理确诊为纤维血管瘤。1例因肿瘤过大而未行切除,3个月后再次栓塞另一侧上颌动脉供血,未再发生鼻出血。结论：三羟甲基-丙烯酸微球可安全地栓塞鼻咽和鼻窦纤维血管瘤,栓塞后肿瘤可缩小,有利于手术切除。     

Clinical emergence of neurometastatic merkel cell carcinoma: a surgical case series and literature review

Merkel cell carcinoma (MCC) is a rare cutaneous neuroendocrine neoplasm of possible viral origin and is known for its aggressive behavior. The incidence of MCC has increased in the last 15 years. Merkel cell carcinoma has the potential to metastasize, but rarely involves the central nervous system. Herein, we report three consecutive surgical cases of MCC presenting at a single institution within 1 year. We used intracavitary BCNU wafers (Gliadel^®) in two cases. Pathological features, including CK20 positivity, consistent with MCC, were present in all cases. We found 33 published cases of MCC with CNS involvement. We suggest that the incidence of neurometastatic MCC may be increasing, parallel to the increasing incidence of primary MCC. We propose a role for intracavitary BCNU wafers in the treatment of intra-axial neurometastatic MCC.     

Juvenile granulosa cell tumor of the testis: case report and review of literature

Juvenile granulosa cell tumor of the testis is an infrequent tumor of the gonadal stroma characteristic of the pediatric age. It usually appears as a scrotal mass and less frequently as an abdominal or inguinal mass. It may be associated with ambiguous genitalia and/or abnormal sex chromosomes. The recommended treatment is orchiectomy alone because local recurrence or metastasis have never been observed. We describe a patient with a juvenile granulosa cell tumor of the testis and review the literature.