scholarly journals Axillary Paget disease with a visible satellite: a case report and literature review

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Wu-Yang Ji ◽  
Bin Luo ◽  
Xue-Wei Wang ◽  
Ying Xiao ◽  
Jin-Yi Tian

Abstract Background Extramammary Paget disease (EMPD) is an uncommon malignancy affecting apocrine gland–bearing skin, such as vulvar, perianal, axillary and penoscrotal areas. Paget cells are sometimes detected outside clinical border in a phenomenon called subclinical extension. Satellite is one of the patterns of subclinical extension which is likely to be invisible. The standard management strategy for EMPD without distant metastasis is a complete surgical removal, sometimes called wide local excision. However, there is no consensus regarding surgical margin width to decrease the high recurrence rate. Here we describe the first macroscopically visible satellite of axillary EMPD and wide local excision of both main lesion and the satellite lesion with only 0.5 cm margin, succeeded by a short review of the literature. Case presentation A 48-year-old female with a red macule in the right axilla was presented to our clinic. A well-demarcated 4 cm × 3 cm erythematous plaque was observed in the right axilla, and a similar lesion measuring 0.5 cm × 0.3 cm was found 3.5 cm away from the primary site. Breast and axillary node examination was unremarkable. Biopsy of the large plaque revealed Paget disease, then we performed a local extended excision of both lesions with a 0.5 cm margin, all margins negative indicated, by frozen pathology. Pathology revealed the nature of the satellite beside the main lesion also as Paget disease. The patient is currently followed-up for 20 months and has shown no signs of recurrence, with normal shoulder motor function. Conclusion We have report the first visible satellite of extramammary Paget disease, indicating the necessity of an extended local resection of both the main leision and the satellite lesion. Considering the anatomical structure of axillary Paget disease, a 0.5 cm negative surgical margin indicated by frozen pathology might be sufficient to sustain the shoulder motor function.

2021 ◽  
Author(s):  
Wuyang Ji ◽  
Bin Luo ◽  
Xue-Wei Wang ◽  
Ying Xiao ◽  
Jin-Yi Tian

Abstract Background : Extramammary Paget disease is an uncommon malignancy affecting apocrine gland–bearing skin, such as vulvar, perianal, axillary and penoscrotal areas. Paget cells are sometimes detected outside the clinical border in a phenomenon called subclinical extension. Satellite is one of the patterns of subclinical extension which is likely to be invisible. The standard management strategy for EMPD without distant metastasis is a complete surgical removal, sometimes called wide local excision. However, there is no consensus regarding surgical margin width to decrease the high recurrence rate. Here we describe the first macroscopically visible satellite of axillary EMPD and wide local excision of both main lesion and the satellite lesion with only 0.5cm margin, succeeded by a short review of the literature. Case presentation: A 48-year-old female with a red macule in the right axilla was presented to our clinic. A well-demarcated 4 cm×3 cm erythematous plaque was observed in the right axilla, and a similar lesion measuring 0.5 cm×0.3 cm was found 3.5 cm away from the primary site. Breast and axillary node examination was unremarkable. Biopsy of the large plaque revealed Paget disease, then we performed a local extended excision of both lesions with a 0.5cm margin, all margins negative indicated, by frozen pathology. Pathology revealed the nature of the satellite beside the main lesion also as Paget disease. The patient is currently followed-up for 20 months and has shown no signs of recurrence, with normal shoulder motor function. Conclusion: We have report the first visible satellite of extramammary Paget disease, indicating the necessity of an extended local resection of both the main leision and the satellite lesion. Considering the anatomical structure of axillary Paget disease, a 0.5cm negative surgical margin indicated by frozen pathology might be sufficient to sustain the shoulder motor function.


2013 ◽  
Vol 2013 ◽  
pp. 1-3 ◽  
Author(s):  
Haider Alyousef ◽  
Elsawi M. Osman ◽  
Mohamed A. Gomha

Introduction. Liposarcoma is a rare pathological entity. By far it is the most common histological subtype of genitourinary sarcomas in adults. Approximately two hundred cases were reported in the literature. We are hereby presenting a case with a typical clinical scenario of paratesticular liposarcoma.Case report. A 75-year-old gentleman presented with a painless right hemiscrotal swelling that was progressively increasing in size over the last 6 years. Testicular tumour markers were negative. Imaging showed a heterogenous mass with fat component. Subsequently he underwent wide local excision that included the paratesticular mass along with the right testicle and all right inguinal canal contents up to the deep inguinal ring with the sparing of right illioinguinal nerve. Histopathological examination showed a well differentiated liposarcoma of the spermatic cord. He remained recurrence-free so far after 18 months of followup.Conclusion. Radical orchidectomy with wide local excision comprises the cornerstone of treatment of paratesticular liposarcoma. Due to the rarity of the disease there is no definite universal consensus of opinion as regards the role of radiotherapy and chemotherapy.


2021 ◽  
Vol 108 (Supplement_7) ◽  
Author(s):  
Katherine Fox ◽  
Lucy Khan

Abstract Backgound Fibromatosis is a rare disease, accounting for 0.2% of breast tumours and 3% of soft tissue tumours. Due to its rarity there is a lack of multi centre trials and therefore evidence and guidance on the best treatment options. Likewise, many clinicians are unfamiliar with how to best manage these patients, even in specialist centres. Methods We present the case of a patient presenting with a suspicious breast lump, diagnosed as fibromatosis. Literature search enabled review of current trends and opinions in the management of such patients. Results We discuss the case of a 70 year old patient with a right breast lump, presenting 3 years following wide local excision and radiotherapy for DCIS. Imaging and examination were suspicious for malignancy. Biopsy demonstrated fibromatosis, for which the patient is currently being managed with active observation. Discussion and Conclusion Desmoid tumours of the breast are a rare but important differential in patients presenting with a breast lump. The aetiology remains poorly understood but they have been linked to genetic conditions, and trauma. As with this case, trauma may be iatrogenic in the form of surgery or radiotherapy. Due to the destructive nature of fibromatosis, the current trend in the management of these patients advocates wide local excision where possible. However, there is a high recurrence rate. There has been some success with medical therapies such as NSAIDs and tyrosine kinase inhibitors. These may be viable options in patients in whom surgery is not suitable.


Author(s):  
Siti Sarah Mohd Ramli ◽  
Asma Abdullah ◽  
Suria Hayati Md Pauzi ◽  
Masturah Ramli

Ceruminous adenoma is described as a glandular neoplasm of ceruminous glands. It is seen for less than one percent of all external ear tumours. Ceruminous adenoma cases were reported to have recurrence and residual tumour, however there was no malignant transformation known in ceruminous adenoma up to this date. Here, we report a young adult woman with two years history of progressive reduced hearing and tinnitus of the right ear. She was proven to have right moderate conductive hearing loss with pure tone audiometry. There was a cystic mass with serous content arising from the posterior wall of the right ear canal. We proceeded with wide local excision of the mass via trans-canal approach. Ceruminous adenoma was confirmed with histopathology and immunohistochemistry of CK7 staining. The hearing impairment was resolved completely post excision and there was no recurrence of the tumour on one year follow-up. We concluded wide local excision with appropriate margin of the mass is adequate to prevent recurrence in ceruminous adenoma cases.


2012 ◽  
Vol 2 (1) ◽  
pp. 30 ◽  
Author(s):  
Ajay H. Bhandarwar ◽  
Girish D. Bakhshi ◽  
Ashok D. Borisa ◽  
Amol Wagh ◽  
Rajat Kapoor ◽  
...  

Ameloblastoma is a benign odontogenic tumor. These are usually asymptomatic until a large size is attained. Ameloblastoma has tendency to spread locally and has a high recurrence rate. Majority of ameloblastomas (80%) arise from the mandible. Ameloblastoma arising from anterior mandibular region (symphysis- menti) is rare. Very few cases of midline anterior ameloblastomas are reported in the literature. They often require wide local excision. Reconstruction of mandible in these cases is challenging. We present a case of mandibular ameloblastoma arising from symphysis- menti. Patient underwent wide surgical excision of the tumor followed by immediate reconstruction using free fibular vascular flap, stabilized with titanium reconstructive plates. A brief case report ands review of literature is presented.


2015 ◽  
Vol 2015 ◽  
pp. 1-4 ◽  
Author(s):  
Adamantia Zizi-Sermpetzoglou ◽  
Despoina Myoteri ◽  
Kalliroi Koulia ◽  
Vassilios Kontostolis ◽  
Hippokratis Moschouris ◽  
...  

Introduction. Aggressive angiomyxoma is a rare, slowly growing, and benign tumour of mesenchymal origin, which affects women of reproductive age and is associated with a high risk of local recurrence.Case Presentation. A case of a 47-year-old white female is presented herein, with a large polypoid, gelatinous mass on the right labia majora, measuring 26 × 21 × 6 cm. Histopathologically, the lesion was composed of spindle and stellate-shaped cells embedded in a myxoid matrix. Another specific feature was the presence of variable-sized thin-walled capillaries and thick-walled vascular channels. The patient underwent wide local excision of the tumour with clear margins and developed local recurrence 18 months later.Discussion. Aggressive angiomyxoma of the vulva needs to be distinguished from benign myxoid tumors with a low risk of local recurrence as well as from malignant myxoid neoplasms. Usually wide local excision with tumour-free margins and occasionally hormonal manipulation is the treatment of choice.


Author(s):  
Christine U. Lee ◽  
James F. Glockner

66-year-old woman with invasive lobular carcinoma and LCIS of the right breast status post wide local excision about 15 months ago followed by radiation therapy The axial subtracted image at peak enhancement with CAD color overlay (Figure 15.4.1) demonstrates postoperative and postradiation changes in the right breast without any worrisome kinetic enhancement. Axial noncontrast water (...


2020 ◽  
Vol 63 (3) ◽  
pp. 406-407
Author(s):  
Ipek Sapci ◽  
Jim Tiernan ◽  
Raffi Gurunian ◽  
Emre Gorgun

2016 ◽  
Vol 6 (1) ◽  
pp. 8
Author(s):  
Gouthaman Shanmugasundaram ◽  
Sivasundari Maharajan

Aggressive Angiomyxoma (AA) is a rare locally aggressive soft tissue tumor with high chance of local recurrence occuring in young women of reproductive age group. These neoplasms are characterized by a mixture of spindle or stellate cells in a loosely myxoid stroma with prominent vascular component containing large, thickwalled blood vessels. A 35 year old multiparous lady presented with right vulval swelling which was rapidly progressing in size. Local examination revealed a soft fleshy mass occupying whole of the right labia majora. In view of the rapidly enlarging lesion over a short duration, we suspected a soft tissue neoplasm and preceded with wide local excision of the mass with adequate margins in all dimensions, eventhough we did not have a preoperative tissue diagnosis. To our surprise, final histopathology was Aggressive Angiomyxoma (AA). The treatment of choice for Aggressive Angiomyxoma is wide local excision with negative margins and the same was done in our case. Recurrence of the tumor may be avoided by wide local excision with adequate margins. It is possibly a hormonally responsive neoplasm. Treatment with Gonadotropin-releasing hormone is an emerging therapy.Overall prognosis of AA is good.


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