scholarly journals Aggressive Angiomyxoma with Perineal Herniation

2014 ◽  
Vol 4 ◽  
pp. 23 ◽  
Author(s):  
Seema Narang ◽  
Supreethi Kohli ◽  
Vinod Kumar ◽  
Raj Chandoke

Aggressive angiomyxoma is a rare mesenchymal tumor involving the pelvic-perineal region. It occurs during the third and fourth decade of life and is predominantly seen in females. It presents clinically as a soft tissue mass in variable locations such as vulva, perianal region, buttock, or pelvis. Assessment of extent of the tumor by radiological evaluation is crucial for surgical planning; however, biopsy is essential to establish diagnosis. We present the radiological and pathological features seen in a 43-year-old female diagnosed with abdominal angiomyxoma with an unusual extension to the perineum.

2019 ◽  
Vol 12 (5) ◽  
pp. e227475 ◽  
Author(s):  
Mee-young Lee ◽  
Brandon da Silva ◽  
Daniel C Ramirez ◽  
Robert G Maki

We describe a case of a 44-year-old woman with locally advanced aggressive angiomyxoma with a novel translocation high-mobility group AT-hook 2–yes-associated protein 1 (HMGA2-YAP1) fusion, implying a t(11;12)(q22.1;q14.3) translocation. She was started on gonadotropin-releasing hormone agonist injection and an aromatase inhibitor for persistent disease, which responded to treatment; she was subsequently treated with radiation before a more definitive operation was conducted. This case report indicates that HGMA2-YAP1–translocated aggressive angiomyxoma is responsive to oestrogen antagonism and hopefully will allow for the development of diagnostics useful for this rare but often morbid neoplasm. This case also highlights the importance of appropriate workup of a soft tissue mass.


2012 ◽  
Vol 2012 ◽  
pp. 1-7 ◽  
Author(s):  
Pratiksha Yadav ◽  
Dolly Thakkar ◽  
S. S. Thind

Primary chondrosarcoma is the third most frequent primary malignancy of bone after myeloma and osteosarcoma. It is ranging from slow growing nonmetastasising lesions to highly aggressive lesions. We report a case of primary intramedullary chondrosarcoma of proximal humerus. A 60-year-old female presented with pain and hard swelling involving the left arm for 5 months. Radiograph showed a lucent expansile intramedullary lesion with matrix calcification and associated soft tissue mass. CT confirmed the finding. MRI showed a lobulated lesion which is hyperintense on T2WI with low signal fibrous septae. Increased tracer uptake was seen on bone scan. Histopathology confirmed the radiology diagnosis. The patient underwent wide resection and endoprosthetic reconstruction of proximal humerus.


2013 ◽  
Vol 12 (5) ◽  
pp. 517-520 ◽  
Author(s):  
Christina Huang ◽  
Elias Rizk ◽  
Mark Iantosca ◽  
Andrea L. Zaenglein ◽  
Klaus F. Helm ◽  
...  

An in utero female was found to have a small hemorrhage at the foramen of Monro, hydrocephalus, and what was originally interpreted as a Dandy-Walker variant. At birth she had macrocephaly and numerous cutaneous, multifocal, red-pink blanchable macules. Postnatal MRI demonstrated a hemorrhagic soft-tissue mass involving the upper brainstem, thalamus, and basal ganglia most consistent with in utero complex multifocal intracranial hemorrhage. The skin lesions were thought to be consistent with multifocal lymphangioendotheliomatosis with thrombocytopenia (MLT). The size and location of the hemorrhage precluded operative intervention, although the hydrocephalus was treated with a ventricular shunt. The child continues to have severe developmental delays. Multifocal lymphangioendotheliomatosis with thrombocytopenia is a multifocal vascular disorder most commonly involving the skin and gastrointestinal tract. Intracranial hemorrhages are rare in this context. This case is the third reported instance of MLT with associated intracranial hemorrhage and the only case described in the neurosurgical literature. The authors review the presenting features and pathophysiology of this condition.


1998 ◽  
Vol 19 (10) ◽  
pp. 698-704 ◽  
Author(s):  
H. Thomas Temple ◽  
Mark S. Mizel ◽  
Mark D. Murphey ◽  
Donald E. Sweet

A total of 329 patients with osteoblastoma were retrospectively reviewed from the archives of the Armed Forces Institute of Pathology, of which 41 (12.5%) presented with tumors in the foot and ankle. This was the third most common site of disease after the spine and femur. Overall, the mean age was 22.5 years, which was the same for the foot and ankle subset of patients; however, there was a significant male predominance in foot and ankle patients compared with the whole group. The majority of patients were skeletally mature (85.4%). Clinically, most patients presented with pain (97.2%), although one-third of the total related a history of antecedent trauma. The interval between the onset of symptoms and biopsy was 84 days (range, 0–572 days). Radiographically, the majority of lesions were in the hindfoot (N = 18; 44%) of which 16 of 18 tumors (89%) were in the talus. Of these, one-half were subperiosteal and dorsally based and were associated with osseous tumor matrix and a soft tissue mass. Two osteoblastomas, both in the metatarsals, transitioned into sarcomas; the rest were histologically benign. For diagnostic purposes, it was essential to obtain clinical, radiographic, and histologic correlation.


Author(s):  
Sunil Vitthalrao Jagtap ◽  
Nitesh Nasre ◽  
Nitin S. Kshirsagar ◽  
Shubham S. Jagtap

Aggressive angiomyxoma is a rare mesenchymal tumor occurring predominantly in the pelviperineal region. Authors present a case of a 70 years female presented with history of distention of lower abdomen with recurrent pain since 6 months. On clinical examination there was suspicion of fibroid. On USG it was reported as huge fleshy, soft tissue mass measuring 20x10x9 cm in lower abdominal flanks and pelvis with vascular pedicle attached to right parametrium. Pan-hysterectomy was performed. Right broad ligament showed mass measuring 18.5x10x6 cm. On histopathological examination it was diagnosed as a case of Aggressive angiomyxoma of broad ligament. We are presenting this case for its extreme rarity, clinicohisto-pathological and radiological findings.


2013 ◽  
Vol 49 (6) ◽  
pp. 394-397 ◽  
Author(s):  
Andrea Weissman ◽  
David Jiménez ◽  
Brian Torres ◽  
Karen Cornell ◽  
Shannon P. Holmes

A 13 yr old female spayed Labrador retriever presented for vulvar bleeding. Abdominal radiographs revealed a soft tissue mass in the ventral pelvic canal. A computed tomography (CT) exam and a CT vaginourethrogram localized the mass to the vagina, helped further characterize the mass, and aided in surgical planning. A total vaginectomy was performed and the histologic diagnosis was leiomyoma. Vaginal tumors make up 1.9–3% of all tumors. Seventy-three percent of vaginal tumors are benign, and 83% of those are leiomyomas. Leiomyomas often have a good long-term prognosis with surgical resection. The diagnostic investigation of this case report utilized a multimodal imaging approach to determine the extent and respectability of the vaginal mass. To the best of the authors’ knowledge, this is the first report describing a CT vaginourethrogram.


2020 ◽  
Vol 3 (3) ◽  
pp. 82-88
Author(s):  
Rana Tashhir ◽  
◽  
Yasin Abeer ◽  

Background: Aggressive angiomyxoma (AA) is a rare mesenchymal neoplasm that commonly occurs in perineal region of females. Case Report: A 17 years female presents with bilateral bizarre vulvar swellings, gradually increasing in size for 2 years, painful for 1 week. Sonographic evaluation revealed bilateral well-defined hypoechoic lesions with internal vascularity and cystic areas. Magnetic resonance imaging (MRI) demonstrated these lesions to be iso-intense to skeletal muscles on T1-weighted images (T1WI) and hyperintense on T2-weighted images (T2WI). These were surgically excised and sent for histopathology that confirmed these lesions to be AA. Discussion: AA is an uncommon neoplasm of mesenchymal origin that arises mainly in the perineal soft tissue of adults. It occurs predominantly in females with a peak incidence in fourth decade. [1] In our case the patient was a teenager. In females it may present as a vulvar nodule, polyp or may even be mistaken for a Bartholin’s or labial cyst. In males it involves analogous sites including inguino-scrotal region. [4] Most of the reported cases include solitary unilateral lesions. Conclusion: Our case is unique because the patient was in her teenage and presented with bilateral vulvar angiomyxomas that showed internal vessels on colour doppler imaging.


2021 ◽  
Vol 33 (2) ◽  
pp. 336-339
Author(s):  
Julia Blakey ◽  
Carmen Jerry ◽  
Ana da Silva ◽  
Simone Stoute

A 7-y-old backyard Leghorn chicken ( Gallus domesticus) was submitted to the California Animal Health and Food Safety Laboratory System (CAHFS)–Turlock branch for postmortem examination, with a history of unexpected death. At postmortem examination, a hemorrhagic soft tissue mass was observed in the cervical region. Microscopically, a densely cellular neoplasm of polygonal epithelial cells and small lymphocytes was observed. The microscopic features of the neoplasm in combination with positive immunohistochemistry for pancytokeratin and CD3 were used to classify the lesion as a thymoma. Thymoma was diagnosed in only 5 birds submitted to CAHFS from 1990 to 2019. Thymoma has been described only rarely in birds, and is an unusual diagnosis in backyard chickens.


VCOT Open ◽  
2021 ◽  
Vol 04 (01) ◽  
pp. e41-e46
Author(s):  
Federica Aragosa ◽  
Chiara Caterino ◽  
Giovanni Della Valle ◽  
Ilaria D'Aquino ◽  
Dario Costanza ◽  
...  

AbstractThe aim of this report is to describe an unusual localization of nerve sheath tumour (NST), clinical presentation, imaging, surgical management, and outcome in a 2-year-old dog. A 2-year-old female American Staffordshire Terrier presented with nonambulatory paraparesis, thoracolumbar hyperaesthesia, hindlimb hyperreflexia, and mild muscle atrophy. Computed tomography and magnetic resonance imaging revealed an extradural mass at T7-T8, without vertebral lesions. Surgical treatment consisted in resection of the soft tissue mass through dorsal laminectomy. The dog was ambulatory within 24 hours and free of recurrence at 18 months postoperatively. Histopathologic and features of immunohistochemistry were consistent with NST. The NST of this report was similar to those described before, but exhibited unusual characteristics, such as being extradural, without extension into intervertebral foramina, and being located in an atypical region (T7-T8). Moreover, survival time and relapse-free interval are greater than previously reported for similar cases.


2021 ◽  
Vol 14 (3) ◽  
pp. e237099
Author(s):  
Daanesh Huned ◽  
Juinn Huar Kam ◽  
Lui Shiong Lee ◽  
Raj Vikesh Tiwari

Synovial sarcomas are most commonly localised in extremities, especially in the lower thigh and knee areas. Comprising less than 1% of all malignancies, retroperitoneal synovial sarcoma is very rare with primary synovial sarcoma of the kidney being even more infrequent and difficult to diagnose. We describe a case report of a renal synovial sarcoma in a young adult who was initially managed as a case of Wunderlich’s syndrome secondary to what was believed to be a ruptured renal angiomyolipoma. After biopsy confirmation, the patient was eventually managed with neo-adjuvant chemotherapy followed by a right radical nephrectomy and right hepatectomy. Despite its rarity, synovial sarcoma should be considered as differential diagnosis of a bleeding retroperitoneal soft tissue mass detected in young adults.


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