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Diagnostic Pathology Mar 2020Malignant eccrine spiradenoma is one of the rarest sweat-gland tumors. Here, we describe a rare case of low grade malignant eccrine spiradenoma located at the vulva. (Review)
Review
BACKGROUND
Malignant eccrine spiradenoma is one of the rarest sweat-gland tumors. Here, we describe a rare case of low grade malignant eccrine spiradenoma located at the vulva.
CASE PRESENTATION
The vulvar lesion was described as a mass measured 3.5 cm and located in the dermis and subcutis with no attachment to the epidermis. The neoplasm was arranged in ragged sheets or solid nodules sometimes with focal necrosis. The tumor cells had hyperchromatism, pleomorphism, and prominent nucleoli with high mitotic index and KI-67 estimated at 70-80%.
CONCLUSIONS
It's only the fifth case of malignant eccrine spiradenoma localized at the vulva. This is the first time that an HPV genotyping was made in this type of lesion with no HPV found while the p16 expression was diffuse. Moreover, it's the first time that a p53 mutation is detected by sequencing in this location.
Topics: Acrospiroma; Biomarkers, Tumor; Breast Neoplasms; Female; Humans; Middle Aged; Mutation; Neoplasms, Second Primary; Papillomaviridae; Sweat Gland Neoplasms; Tumor Suppressor Protein p53; Vulvar Neoplasms
PubMed: 32169099
DOI: 10.1186/s13000-020-00945-9 -
Medicine Nov 2022Adenoid cystic carcinoma (ACC) often occurs in the major and minor salivary glands and other sites containing secretory glands, while ACC of the Bartholin's gland... (Review)
Review
RATIONALE
Adenoid cystic carcinoma (ACC) often occurs in the major and minor salivary glands and other sites containing secretory glands, while ACC of the Bartholin's gland (BG-ACC) in the vulva is rare and easily misdiagnosed.
PATIENT CONCERNS
A 58-year-old female was referred to our hospital for further valuation of a mass occurring on the left side of her vulva. In the other hospital, the beginning of the period, local ultrasound showed a vulva mass, which was suspected to be a Bartholin's gland cyst. Mixed neoplasms were considered in some biopsies. When transferred to our hospital, virtuous tumors were considered by ultrasound and magnetic resonance imaging. Pathology initially considered benign hyperplastic active tumor or borderline tumor.
DIAGNOSES
Histological, immunochemical, and molecular tests confirmed a diagnosis of BG-ACC, negative surgical margin, without lymphatic metastasis.
INTERVENTIONS
Extended excision of the mass at left labia majora plus left inguinal lymph node dissection was performed.
OUTCOMES
The patient received surgery therapy, no recurrence was observed during a 18-month follow-up period.
LESSONS
Due to its lack of specific characteristics in clinical, ultrasound and imaging, it is easy to be misdiagnosed, Due to its rarity and nonspecific clinical, radiologic and ultrasonographic manifestations, BG-ACC can be easily misdiagnosed. And its pathomorphological features overlap with other benign and malignant tumors occurring at vulva, BG-ACC can be easily misdiagnosed, and diagnosis by puncture biopsy is extremely difficult. Use of paraffin sections to identify tumor growth characteristics, combined with immunohistochemical findings, is the key to the diagnosis of ACC. In rare sites, MYB gene split are helpful in making a definite diagnosis.
Topics: Humans; Female; Middle Aged; Bartholin's Glands; Carcinoma, Adenoid Cystic; Vulvar Neoplasms; Vulva; Diagnostic Errors
PubMed: 36397326
DOI: 10.1097/MD.0000000000031744 -
The Journal of International Medical... Aug 2023Aggressive angiomyxoma is a rare benign tumor with diverse clinical and radiologic presentations, making its differential diagnosis challenging. Here we report the...
Aggressive angiomyxoma is a rare benign tumor with diverse clinical and radiologic presentations, making its differential diagnosis challenging. Here we report the second recurrence of an aggressive angiomyxoma of the vulva in a 33-year-old woman who presented with pain and swelling. Clinical examination revealed a well-circumscribed large palpable mass extending from the inferior part of the right labia major into the right side of the gluteus. A second recurrence of aggressive angiomyxoma was suspected based on the patient's history, clinical examination, and magnetic resonance imaging report, and the patient underwent surgical resection with a negative margin. Histological evaluation of the extracted lesion confirmed the diagnosis of aggressive angiomyxoma. At the 1-year follow-up, the patient was recurrence-free. The present report urges more awareness regarding the aggressiveness of angiomyxoma of the vulva. Closer attention should be given to margin-free removal of such tumors, and patients should be routinely followed up for at least 2 years postoperatively for early diagnosis of recurrence, thereby reducing the risk of morbidity.
Topics: Female; Humans; Adult; Vulvar Neoplasms; Vulva; Myxoma; Diagnosis, Differential
PubMed: 37551697
DOI: 10.1177/03000605231189366 -
World Journal of Surgical Oncology Apr 2015Schwannoma is a benign, solitary, slow-growing neoplasm of the peripheral nerve sheath. These tumors are rarely found in the external genital system, and only a few...
Schwannoma is a benign, solitary, slow-growing neoplasm of the peripheral nerve sheath. These tumors are rarely found in the external genital system, and only a few cases of vulvar schwannoma have been reported. Herein, we report a case of a vulvar schwannoma. A 37-year-old woman presented with a 3-cm-sized painless mass of the vulva which had been present for 3 years. Magnetic resonance imaging (MRI) of the pelvis showed an isolated finding of a 4.6-cm-sized round mass with a well-defined margin in the midline vulvar area. Simple excision of the tumor was undertaken, and histological examination with immunohistochemical testing demonstrated a vulvar schwannoma. Although benign schwannoma only rarely occurs in the vulva and other external areas of female genitalias, we suggest that it should be considered a differential diagnosis for patients that present a vulvar enlargement or mass. Simple surgical resection and follow-up is the most convenient treatment.
Topics: Adult; Diagnosis, Differential; Female; Humans; Magnetic Resonance Imaging; Neurilemmoma; Prognosis; Vulvar Neoplasms
PubMed: 25879436
DOI: 10.1186/s12957-015-0556-z -
Annales de Dermatologie Et de... Jan 2001Basal cell carcinoma (BCC) accounts for 2 to 3 p. 100 of all vulvar malignancies. (Review)
Review
INTRODUCTION
Basal cell carcinoma (BCC) accounts for 2 to 3 p. 100 of all vulvar malignancies.
PATIENTS AND METHODS
We report a retrospective study of 21 cases treated from 1937 to 1999.
RESULTS
Vulvar BCC's occurred in elderly patients (average age: 66 years), with mean delay to diagnosis of 5.5 years. Five patients were referred for recurrence. Lesions were located on the external hairy side of the labia majus, except one located on the internal side. In 4 cases a preexisting risk factor was identified: 2 patients had previously received radiation therapy and 2 other patients had multiple disseminated BCC. Mean BCC diameter was 2 cm. Pathological data were similar to skin BCC, with one case of mixed tumor (BCC and squamous cell carcinoma). The treatment was surgical excision for 19 BCCs. Local recurrence risk was high. Only one patient died of visceral dissemination of the disease.
DISCUSSION
More than 250 cases of vulvar BCC have been reported in the literature. Clinical, pathological and follow up data are similar to results in the present series. Treatment of choice consists of surgical excision with tumor-free margins. Because of local recurrence risk and possible association with other primary cancers in this age group, long term follow-up is necessary.
Topics: Adult; Aged; Aged, 80 and over; Carcinoma, Basal Cell; Female; Humans; Retrospective Studies; Vulvar Neoplasms
PubMed: 11226893
DOI: No ID Found -
Taiwanese Journal of Obstetrics &... Jan 2021To report a case of vulvar epithelioid sarcoma, a rare type of tumor that has been reported in 37 cases in the English literature to date. (Review)
Review
OBJECTIVE
To report a case of vulvar epithelioid sarcoma, a rare type of tumor that has been reported in 37 cases in the English literature to date.
CASE REPORT
We report three cases of vulvar epithelioid sarcoma, proximal type. Wide excisions of the mass were performed with margins free of tumor in all three cases.
CONCLUSION
Due to its low incidence, there are no evidence-based diagnostic algorithms or published recommendations for treatment. Locoregional lymph node involvement, vascular invasion, tumor size larger than 2 cm, deep localization, presence of necrosis, and a high mitotic index are known as poor prognostic factors. Adjuvant radiotherapy is advisable in the presence of a high-grade tumor or positive margins. The beneficial effect of adjuvant chemotherapy is not well established. Treatment decisions should be made based on the individual case presentation and pathology evaluation.
Topics: Adult; Female; Humans; Medical Illustration; Sarcoma; Vulva; Vulvar Neoplasms; Young Adult
PubMed: 33494986
DOI: 10.1016/j.tjog.2020.11.020 -
Acta Obstetricia Et Gynecologica... Jan 2023In the Netherlands, the sentinel lymph node procedure protocol consists of preoperative lymphoscintigraphy combined with intraoperative blue dye for identifying sentinel...
INTRODUCTION
In the Netherlands, the sentinel lymph node procedure protocol consists of preoperative lymphoscintigraphy combined with intraoperative blue dye for identifying sentinel lymph nodes in early vulvar squamous cell carcinoma. This study aimed at investigating the role of early and late lymphoscintigraphy.
MATERIAL AND METHODS
From January 2015 to January 2019, early and late lymphoscintigraphies of 52 women were retrospectively analyzed. Lymphoscintigraphy was performed 30 minutes (early) and 2.5-4 hours (late) after vulvar injection of Tc-labeled nanocolloid. We calculated the concordance correlation coefficient (CCC) between number of sentinel lymph nodes detected on both images using the Lins concordance coefficient and correlated with clinicopathological data.
RESULTS
Thirty-four women had a midline tumor and 18 had a lateral tumor. Detection rates with early and late scintigraphy were 88.5% and 98.1%, respectively. Median number of detected nodes was 1.0 (0-7) and 2.0 (0-7). Good statistical correlation between number of sentinel lymph nodes detected on early and late imaging was found (CCC = 0.76) in most patients. In 18 women (35%) a mismatch occurred: a higher number of nodes was detected on late imaging. In 11 of 18 women re-injection was performed because no sentinel lymph nodes were visualized on early images. Late imaging and intraoperative detection showed a good statistical correlation (CCC = 0.61). One woman showed an isolated groin recurrence despite negative sentinel lymph nodes.
CONCLUSIONS
This study showed good statistical correlations between early and late scintigraphy in most patients. However, in 35% of women late scintigraphy detected more nodes. In case of poor visualization after the first scintigraphy, re-injection should be considered. Late scintigraphy is probably helpful in confirming successful re-injection and in showing deviating lymph flow in women with failed mapping after the first injection and successful re-injection. Because missing metastatic sentinel lymph nodes often leads to a poor prognosis, we prefer optimal correlations between imaging and intraoperative identification. Hence, late scintigraphy cannot be safely omitted.
Topics: Humans; Female; Lymphoscintigraphy; Lymph Nodes; Sentinel Lymph Node Biopsy; Vulvar Neoplasms; Retrospective Studies; Lymphatic Metastasis; Radiopharmaceuticals
PubMed: 36412099
DOI: 10.1111/aogs.14476 -
European Journal of Nuclear Medicine... Sep 2021This retrospective study aimed to assess the diagnostic performance of preoperative [F]FDG-PET/CT in predicting the groin and pelvic lymph node (LN) status in a large...
PURPOSE
This retrospective study aimed to assess the diagnostic performance of preoperative [F]FDG-PET/CT in predicting the groin and pelvic lymph node (LN) status in a large single-centre series of vulvar cancer patients.
METHODS
Between January 2013 and October 2018, among all consecutive women with proven vulvar cancer submitted to [F]FDG-PET/CT, 160 patients were included. LNs were analysed by two qualitative methods assessing PET information (defined as visual assessment) and a combination of PET and low-dose CT information (defined as overall assessment), respectively, as well as semi-quantitative analysis (LN-SUV). Sensitivity, specificity, accuracy, positive and negative predictive values (PPV and NPV) in predicting the groin and pelvic LN status were calculated in the overall study population; a subset analysis of groin parameters in clinically/ultrasonography negative patients was also performed. Histopathology was the reference standard.
RESULTS
All patients underwent vulvar and inguinofemoral LN surgery, and 35 pelvic LN surgery. Overall, 338 LN sites (296 groins and 42 pelvic sites) were histologically examined with 30.4% prevalence of metastatic groins and 28.6% for metastatic pelvic sites. In the overall study population, sensitivity (95% confidence interval, CI), specificity (95% CI), accuracy (95% CI), PPV (95% CI) and NPV (95% CI) at the groin level were 85.6% (78.3-92.8), 65.5% (59.0-72.0), 71.6% (66.5-76.8), 52.0% (44.0-60.1) and 91.2% (86.7-95.8) for visual assessment; 78.9% (70.5-87.3), 78.2% (72.5-83.8), 78.4% (73.7-83.1), 61.2% (52.3-70.1) and 89.4% (85.0-93.9) for overall assessment; and 73.3% (64.2-82.5), 85.0% (80.1-89.8), 81.4% (77.0-85.8), 68.0% (58.8-77.3) and 87.9% (83.4-92.5) for semi-quantitative analysis (SUV cut-off value 1.89 achieved by ROC analysis). Similar results were observed in the pelvis-based analysis.
CONCLUSION
In this large single-centre series of vulvar cancer patients, [F]FDG-PET/CT showed good values of sensitivity and NPV in discriminating metastatic from non-metastatic LNs. In routine clinical practice, qualitative analysis is a reliable interpretative criterion making unnecessary commonly used semi-quantitative methods such as SUV.
Topics: Female; Fluorodeoxyglucose F18; Humans; Lymph Nodes; Lymphatic Metastasis; Neoplasm Staging; Positron Emission Tomography Computed Tomography; Radiopharmaceuticals; Retrospective Studies; Sensitivity and Specificity; Vulvar Neoplasms
PubMed: 33619601
DOI: 10.1007/s00259-021-05257-8 -
Journal of Cancer Research and... 2019The aim of this study was to evaluate the patterns of recurrence and outcomes in patients with vulvar cancer treated with surgery, with or without postoperative...
AIM
The aim of this study was to evaluate the patterns of recurrence and outcomes in patients with vulvar cancer treated with surgery, with or without postoperative radiotherapy (RT) or RT alone.
MATERIALS AND METHODS
The hospital records were retrospectively analyzed from February 2007 to May 2016. All patients that had biopsy-proven carcinoma vulva were included for analysis. Surgery was done by simple or radical vulvectomy with or without lymph node dissection. Radical RT dose was 60 Gy (photons alone or with electron boost). Adjuvant RT was administered in case of node positivity or positive margins.
RESULTS
A total of 76 patients were diagnosed as squamous cell carcinoma of vulva. The median age was 59 years. About 59 patients were treated by surgery and 17 patients were treated by radical RT. The most common stage was IB. Adjuvant RT was administered in 10 patients. About 13 patients (22%) underwent inguinal lymphadenectomy. At a median follow-up of 35 months, 49 patients (64.4%) were alive, 27 died, and 6 patients were lost to follow-up. Locoregional recurrence rate was 24.5% in radical surgery group, 12% in surgery plus adjuvant RT group, and 47% in radical RT group. Distant metastasis rate was 4%. The progression-free survival at 3 years was 45.3% and 35.5% in the surgery group and radical RT group, respectively.
CONCLUSION
Surgery resulted in favorable local control rates in early-stage carcinoma vulva. Adjuvant RT is indicated in case of nodal positivity or positive margins. Radical RT can be considered as an alternative in patients with comorbidities.
Topics: Adult; Aged; Aged, 80 and over; Carcinoma; Combined Modality Therapy; Female; Histocytochemistry; Humans; Middle Aged; Neoplasm Recurrence, Local; Treatment Outcome; Vulvar Neoplasms
PubMed: 31898668
DOI: 10.4103/jcrt.JCRT_44_18 -
Journal of Gynecologic Oncology Nov 2023Extramammary Paget's disease (EMPD) of the vulva is a rare disease which predominantly presents in postmenopausal Caucasian women. As yet, no studies on Asian female...
OBJECTIVE
Extramammary Paget's disease (EMPD) of the vulva is a rare disease which predominantly presents in postmenopausal Caucasian women. As yet, no studies on Asian female patients with EMPD have been performed. This study aimed to identify the clinical features of patients with vulvar EMPD in Korea, and to evaluate the risk factors of recurrence and postoperative complications in surgically treated EMPD.
METHODS
We retrospectively reviewed 47 patients with vulvar EMPD who underwent wide local excision or radical vulvectomy. The clinical data and surgical and oncological outcomes following surgery were extracted from medical records and analyzed. Univariate and multivariate analyses for predicting recurrence and postoperative complications were performed.
RESULTS
21.3% of patients had complications after surgery, and wound dehiscence was the most common. 14.9% of patients experienced recurrence, and the median interval to recurrence from initial treatment was 69 (range 33-169) months. Vulvar lesions larger than 40 mm was the independent risk factor of postoperative complications (odds ratio [OR]=7.259; 95% confidence interval [CI]=1.545-34.100; p=0.012). Surgical margin status was not associated with recurrence in surgically treated vulvar EMPD patients (OR=0.83; 95% CI=0.16-4.19; p=1.000).
CONCLUSION
Positive surgical margin is a frequent finding in the patients with vulvar EMPD, but disease recurrence is not related with surgical margin status. Since EMPD is a slow growing tumor, a surveillance period longer than 5 years is required.
Topics: Humans; Female; Paget Disease, Extramammary; Prognosis; Retrospective Studies; Margins of Excision; Neoplasm Recurrence, Local; Vulva; Vulvar Neoplasms; Postoperative Complications
PubMed: 37477099
DOI: 10.3802/jgo.2023.34.e76