BACKGROUND Aggressive angiomyolipoma is an extremely rare benign mesenchymal tumor that was originally described as a locally recurrent mucinous spindle cell tumour.Agg-ressive angiomyolipoma originates from myofibrob...BACKGROUND Aggressive angiomyolipoma is an extremely rare benign mesenchymal tumor that was originally described as a locally recurrent mucinous spindle cell tumour.Agg-ressive angiomyolipoma originates from myofibroblasts,vascular smooth muscle cells,or fibroblasts,and displays various phenotypes of myofibroblasts and abnor-mal muscle arteries.Aggressive angiomyolipoma was first identified in 1983 and fewer than 50 male patients have been reported to date.It is an extremely rare mesenchymal tumour and often confused with other diseases.Patients with epididymal aggressive angiomyolipoma lack typical symptoms,most of which occur incidentally,although some patients may experience mild pain,discomfort,and swelling.Pain may be exacerbated by pressure from the mass.CASE SUMMARY A 66-year-old male was admitted to the hospital on January 14,2022 with chief complaint of swelling in the left scrotum for one year.There was no apparent cause for the swelling.The patient did not consult with any doctor or receive any treatment for the swelling.The enlarged scrotum increased in size gradually until it reached approximately the size of a goose egg,and was accompanied by discom-fort and swelling of the left cavity of the scrotum.The patient had no history of any testicular trauma,infection,or urinary tract infection.The patient urinated freely,1-2 times at night,without urgency,dysuria(painful urination),or haematuria.There was no significant family history of malignancy.The patient underwent excision of the enlarged tumour and the left epididymis under general anaes-thesia on January 18,2022.Twelve months of follow-up revealed no recurrence.The patient was satisfied with the treatment.CONCLUSION Aggressive angiomyolipoma is extremely rare clinically and often confused with other diseases.The pathogenesis of aggressive angiomyolipoma is unclear and the clinical presentation is mostly a painless enlarged mass.The diagnosis of aggressive angiomyolipoma requires a combination of medical history,preoperative imaging such as computed tomography and magnetic resonance imaging,cytological examination and preoperative and postoperative pathological biopsy.The preferred treatment is surgery,with the possibility of a new alternative treatment option after hormonal therapy.Aggressive angiomyolipoma should be considered in the differential diagnosis of parametrial tumors of the male genital area that present as clinically significant masses.The high recurrence rate of aggressive angiomyolipoma may be related to incomplete tumor resection,and patients with aggressive angiomyolipoma are advised to undergo annual postoperative follow-up and imaging for recurrence.展开更多
Objective To explore the clinicopathologic features of angiomyofibroblastomz and the relationship between angiomyofibroblastoma (AMFB)and aggressive angiomyxoma (AA ). Aethods Seven cases of AMFB and 5 coses of AA wer...Objective To explore the clinicopathologic features of angiomyofibroblastomz and the relationship between angiomyofibroblastoma (AMFB)and aggressive angiomyxoma (AA ). Aethods Seven cases of AMFB and 5 coses of AA were reviewed. Conventional histologic and immunohistochemical features of AMFB were compered with those of AA. Results The lesions of AMFB usually presented as painless masses and were located in the superficial vulvar regional and inguinal areas. Microscopically, the tumors were composed of spindled and epithelioid cells arranged in cords and nests preferentially arrayed around numerous small to medium-sized, thin-walled vessels with some hyalined thick walls. Seven cases manifested zone of both dense and sparse cellularity. Immunohistochemically, the tumor cells were strongly positive for vimentin and desmin. but negative for S-100 and cytokeratin 8. Some cells were positive for SMA, ER and PR. None of the 4 ams followed up over 2 years after simple excision developed a recurrence, in contract to AA that exhibited local recurrence in 3 of 4 cases. AA showed more dense myxoid hackground and thick walled vessels. Myoid bundles tended to be located adjacently to biood vessels in all cases. Ultrastructurally,AMFB tumor cells contained intercellular junction and cytoplastic microfilaments. Intercellular spaces contained a great of collagen fibers. Conclusion The results indicate that conventional morphologic analysis is paramount in the recognition of ANFB. AMFB and AA show different clinicoathologic entity and propose an origin from a perivascular stem cell that is ca- pable of myofibroblastic differentiation.展开更多
Aggressive angiomyxoma was first described in 1983 by Steeper and Rosai, and fewer than 150 cases have been reported in the world medical literature. It is a soft-tissue tumour of the pelvis and perineum. The recurren...Aggressive angiomyxoma was first described in 1983 by Steeper and Rosai, and fewer than 150 cases have been reported in the world medical literature. It is a soft-tissue tumour of the pelvis and perineum. The recurrence rate is high, and often extensive resections are performed with considerable morbidity. These tumours are benign, locally infiltrative mesenchymal neoplasms with a predilection for the female pelvis and perineum and they usually tend to recur. Furthermore, these tumours often reach too large dimensions before becoming clinically symptomatic;their incidence is higher in women of the reproductive age group;however a few cases of its occurrence outside the pelvis have also been reported. In this study, we reported three cases with aggressive pelvic angiomyxoma treated with surgical methods and used an approach that described by Kraske in order to get access to lower rectal cancers. Accurate preoperative diagnosis should alert the surgeon to the need for wide excision, which is essential for prevention of local recurrence.展开更多
Background: Ischiorectal Fossa Tumors are rare and their management has not been protocolized. Patients: We retrospectively review four consecutive cases treated in our department, from January 2015 to July 2015. All ...Background: Ischiorectal Fossa Tumors are rare and their management has not been protocolized. Patients: We retrospectively review four consecutive cases treated in our department, from January 2015 to July 2015. All of them were discussed in a multidisciplinary team meeting. None of them were secondary to an inflammatory process. Results: A Magnetic Resonance Imaging was performed in all the four patients, and as it was not a malignant diagnosis made in any of them, we proceeded to resecate the lesions. None biopsies were performed and the benign diagnoses were confirmed by the pathologist. All the patients underwent a local posterior or perineal approach, because all the lesions (epidermoid cyst, hamartoma, lipoma and an aggressive angiomyxoma) were localized purely in the ischiorectal fossa, under the levator ani muscle and not invading any adjacent structures. In all of them, an R0 resection was performed. Conclusions: Ischiorectal fossa tumors are rare and there are few cases already published. Non-inflammatory lesions located in the ischiorectal fossa, with none invasion of rectum or levator ani muscle, are mainly benign lesions prone to their complete excision by a posterior approach. Biopsy is not always necessary unless there’s a suspicion of a malignancy or invasion of adjacent structures, and only in that case a biopsy should be made, because in some of them, a neo-adjuvant treatment can be useful to reduce the tumor and to perform an R0 resection.展开更多
文摘BACKGROUND Aggressive angiomyolipoma is an extremely rare benign mesenchymal tumor that was originally described as a locally recurrent mucinous spindle cell tumour.Agg-ressive angiomyolipoma originates from myofibroblasts,vascular smooth muscle cells,or fibroblasts,and displays various phenotypes of myofibroblasts and abnor-mal muscle arteries.Aggressive angiomyolipoma was first identified in 1983 and fewer than 50 male patients have been reported to date.It is an extremely rare mesenchymal tumour and often confused with other diseases.Patients with epididymal aggressive angiomyolipoma lack typical symptoms,most of which occur incidentally,although some patients may experience mild pain,discomfort,and swelling.Pain may be exacerbated by pressure from the mass.CASE SUMMARY A 66-year-old male was admitted to the hospital on January 14,2022 with chief complaint of swelling in the left scrotum for one year.There was no apparent cause for the swelling.The patient did not consult with any doctor or receive any treatment for the swelling.The enlarged scrotum increased in size gradually until it reached approximately the size of a goose egg,and was accompanied by discom-fort and swelling of the left cavity of the scrotum.The patient had no history of any testicular trauma,infection,or urinary tract infection.The patient urinated freely,1-2 times at night,without urgency,dysuria(painful urination),or haematuria.There was no significant family history of malignancy.The patient underwent excision of the enlarged tumour and the left epididymis under general anaes-thesia on January 18,2022.Twelve months of follow-up revealed no recurrence.The patient was satisfied with the treatment.CONCLUSION Aggressive angiomyolipoma is extremely rare clinically and often confused with other diseases.The pathogenesis of aggressive angiomyolipoma is unclear and the clinical presentation is mostly a painless enlarged mass.The diagnosis of aggressive angiomyolipoma requires a combination of medical history,preoperative imaging such as computed tomography and magnetic resonance imaging,cytological examination and preoperative and postoperative pathological biopsy.The preferred treatment is surgery,with the possibility of a new alternative treatment option after hormonal therapy.Aggressive angiomyolipoma should be considered in the differential diagnosis of parametrial tumors of the male genital area that present as clinically significant masses.The high recurrence rate of aggressive angiomyolipoma may be related to incomplete tumor resection,and patients with aggressive angiomyolipoma are advised to undergo annual postoperative follow-up and imaging for recurrence.
文摘Objective To explore the clinicopathologic features of angiomyofibroblastomz and the relationship between angiomyofibroblastoma (AMFB)and aggressive angiomyxoma (AA ). Aethods Seven cases of AMFB and 5 coses of AA were reviewed. Conventional histologic and immunohistochemical features of AMFB were compered with those of AA. Results The lesions of AMFB usually presented as painless masses and were located in the superficial vulvar regional and inguinal areas. Microscopically, the tumors were composed of spindled and epithelioid cells arranged in cords and nests preferentially arrayed around numerous small to medium-sized, thin-walled vessels with some hyalined thick walls. Seven cases manifested zone of both dense and sparse cellularity. Immunohistochemically, the tumor cells were strongly positive for vimentin and desmin. but negative for S-100 and cytokeratin 8. Some cells were positive for SMA, ER and PR. None of the 4 ams followed up over 2 years after simple excision developed a recurrence, in contract to AA that exhibited local recurrence in 3 of 4 cases. AA showed more dense myxoid hackground and thick walled vessels. Myoid bundles tended to be located adjacently to biood vessels in all cases. Ultrastructurally,AMFB tumor cells contained intercellular junction and cytoplastic microfilaments. Intercellular spaces contained a great of collagen fibers. Conclusion The results indicate that conventional morphologic analysis is paramount in the recognition of ANFB. AMFB and AA show different clinicoathologic entity and propose an origin from a perivascular stem cell that is ca- pable of myofibroblastic differentiation.
文摘Aggressive angiomyxoma was first described in 1983 by Steeper and Rosai, and fewer than 150 cases have been reported in the world medical literature. It is a soft-tissue tumour of the pelvis and perineum. The recurrence rate is high, and often extensive resections are performed with considerable morbidity. These tumours are benign, locally infiltrative mesenchymal neoplasms with a predilection for the female pelvis and perineum and they usually tend to recur. Furthermore, these tumours often reach too large dimensions before becoming clinically symptomatic;their incidence is higher in women of the reproductive age group;however a few cases of its occurrence outside the pelvis have also been reported. In this study, we reported three cases with aggressive pelvic angiomyxoma treated with surgical methods and used an approach that described by Kraske in order to get access to lower rectal cancers. Accurate preoperative diagnosis should alert the surgeon to the need for wide excision, which is essential for prevention of local recurrence.
文摘Background: Ischiorectal Fossa Tumors are rare and their management has not been protocolized. Patients: We retrospectively review four consecutive cases treated in our department, from January 2015 to July 2015. All of them were discussed in a multidisciplinary team meeting. None of them were secondary to an inflammatory process. Results: A Magnetic Resonance Imaging was performed in all the four patients, and as it was not a malignant diagnosis made in any of them, we proceeded to resecate the lesions. None biopsies were performed and the benign diagnoses were confirmed by the pathologist. All the patients underwent a local posterior or perineal approach, because all the lesions (epidermoid cyst, hamartoma, lipoma and an aggressive angiomyxoma) were localized purely in the ischiorectal fossa, under the levator ani muscle and not invading any adjacent structures. In all of them, an R0 resection was performed. Conclusions: Ischiorectal fossa tumors are rare and there are few cases already published. Non-inflammatory lesions located in the ischiorectal fossa, with none invasion of rectum or levator ani muscle, are mainly benign lesions prone to their complete excision by a posterior approach. Biopsy is not always necessary unless there’s a suspicion of a malignancy or invasion of adjacent structures, and only in that case a biopsy should be made, because in some of them, a neo-adjuvant treatment can be useful to reduce the tumor and to perform an R0 resection.
