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International Journal of Environmental... Nov 2022We report a case of a 76-year-old female with a stage IB, grade I endometrioid endometrial carcinoma who presented with right-hip pain and an enlarging black, exophytic,...
CASE
We report a case of a 76-year-old female with a stage IB, grade I endometrioid endometrial carcinoma who presented with right-hip pain and an enlarging black, exophytic, subungual lesion on her right-small-finger distal phalanx. Clinically, the distal phalanx lesion was suspicious for a subungual melanoma; however, advanced imaging suggested metastatic disease, with lesions in the acetabulum, lungs, brain, vulva, and vagina.
CONCLUSION
Partial amputation of the right, small finger and vulvar biopsies confirmed an endometrial carcinoma. To our knowledge, this is the first described case of endometrial adenocarcinoma metastasis to the phalanx of an upper extremity, mimicking a subungual melanoma.
Topics: Humans; Female; Aged; Carcinoma, Endometrioid; Melanoma; Endometrial Neoplasms; Adenocarcinoma; Nail Diseases
PubMed: 36361369
DOI: 10.3390/ijerph192114494 -
Cancers Oct 2022Malignant melanoma is a fatal disease that affects all skin sites. Among these, vulvar melanoma (VM) is a rare gynecological condition that accounts for 5% of all vulvar... (Review)
Review
Malignant melanoma is a fatal disease that affects all skin sites. Among these, vulvar melanoma (VM) is a rare gynecological condition that accounts for 5% of all vulvar neoplasms. VM primarily affects older Caucasian women and its relationship to sun exposure is undefined. Diagnosis is defined by biopsy but many clinical, dermatoscopic, and confocal microscopic features can guide doctors. The molecular profile is characterized by the KIT mutation, revealed by all of the technologies that are used (classical sequencing, next-generation sequencing, and immunohistochemical staining). BRAF and NRAS mutations are also common in VM. All of these mutations are possible therapeutic targets. Today, surgery remains the first treatment choice for primary VM. The role of neoadjuvant and adjuvant therapy is scarce and the treatment of relapses is widely debated.
PubMed: 36358637
DOI: 10.3390/cancers14215217 -
Chirurgie (Heidelberg, Germany) Feb 2023Lymphadenectomy is a surgical procedure in which lymph nodes are surgically resected. It is usually carried out for oncological surgical treatment of various malignant... (Review)
Review
Lymphadenectomy is a surgical procedure in which lymph nodes are surgically resected. It is usually carried out for oncological surgical treatment of various malignant diseases. These include carcinomas of the breast, penis, vulva and anus as well as malignant melanomas and the broad field of sarcomas. A distinction is made between the removal of regional lymph nodes, the sentinel lymph node and the radical removal of lymph nodes in a body region. Cervical, axillary, inguinal and iliac lymph nodes are clinically relevant. The strategy of sentinel lymph node dissection, in which the first lymph node in the drainage system is resected and histopathologically examined for malignant tissue, has brought decisive advantages for the patients, as radical lymphadenectomy with its severe morbidities is utilized in fewer cases. This can improve the patient's quality of life by sparing the lymphatic drainage pathways and reducing lymphedema, inflammation and wound healing disorders. In addition, a lymphadenectomy may be indicated as part of palliative interventions. Another form of lymph node removal is the vascularized lymph node transplantation, which is used for reconstructive purposes in lymphedema. Therefore lymph node grafts are transferred to the site where lymph nodes were previously removed. This review presents the current status of lymphadenectomy in accordance with the German guidelines, anatomical knowledge and specific indications for axillary, inguinal and iliac lymphadenectomy. In addition, an overview of vascularized lymph node transfer is given.
Topics: Male; Female; Humans; Quality of Life; Lymphatic Metastasis; Lymph Nodes; Lymph Node Excision; Lymphedema
PubMed: 36319745
DOI: 10.1007/s00104-022-01739-z -
The Australasian Journal of Dermatology Feb 2023
Review
Topics: Humans; Female; Lichen Sclerosus et Atrophicus; Immune Checkpoint Inhibitors; Vulvar Lichen Sclerosus
PubMed: 36310527
DOI: 10.1111/ajd.13941 -
Cancers Oct 2022Cutaneous melanoma is a relatively common neoplasm, with fairly well understood pathogenesis, risk factors, prognosis and therapeutic protocols. The incidence of this... (Review)
Review
Cutaneous melanoma is a relatively common neoplasm, with fairly well understood pathogenesis, risk factors, prognosis and therapeutic protocols. The incidence of this disease is increasing every year. The situation is different for rare malignancies such as vulvar melanomas and for the even rarer vaginal melanomas. The risk factors for vulvovaginal tumors are not fully understood. The basis of treatment in both cases is surgical resection; however, other types of treatments such as immunotherapy are available. This paper focuses on comparing the pathogenesis and risk factors associated with these neoplasms as well as the efficacy of two groups of drugs-anti-PD-L1 and anti-CTLA4 inhibitors-against both cutaneous melanoma and melanoma of the lower genital tract (vulva and vagina). In the case of cutaneous melanoma, the situation looks more optimistic than for vulvovaginal melanoma, which has a much worse prognosis and, as it turns out, shows a poorer response to immune therapy.
PubMed: 36291906
DOI: 10.3390/cancers14205123 -
Bladder Cancer (Amsterdam, Netherlands) 2022Mucosal melanoma involving the urethra is a rare disease with distinct clinical and molecular characteristics and poor outcomes. Our current knowledge is limited by the...
BACKGROUND
Mucosal melanoma involving the urethra is a rare disease with distinct clinical and molecular characteristics and poor outcomes. Our current knowledge is limited by the small number of reports regarding this disease.
OBJECTIVE
To describe the clinical, pathological, and molecular characteristics of urethral melanoma.
METHODS
We summarized the clinicopathologic data for 31 patients treated for urethral melanoma from 1986-2017 at our institution. Genomic data from our institutional sequencing platform MSK-IMPACT ( = 5) and gene-specific PCR data on , , and/or ( = 8) were compared to genomic data of cutaneous melanomas ( = 143), vulvar/vaginal melanomas ( = 24), and primary non-melanoma urethral tumors ( = 5) from our institutional database.
RESULTS
Twenty-three patients were diagnosed with localized disease, 7 had regional/nodal involvement and one had metastases. Initial treatment included surgery in 25 patients; seven had multimodal treatment. Median follow-up was 46 months (IQR 33-123). Estimated 5-year cancer-specific survival was 45%. No significant change in survival was observed based on a year of treatment.Primary urethral melanomas showed a higher frequency of mutations compared to cutaneous (80.0% vs. 18.2%, = 0.006) and vulvar/vaginal melanomas (80.0 vs. 25.0%, = 0.04). mutations were absent in urethral primaries (0% vs. 46% in cutaneous melanoma, = 0.02). Tumor mutation burden was higher in cutaneous than urethral melanomas ( = 0.04). Urethral melanomas had a higher number of somatic alterations compared to non-melanoma urethral tumors (median 11 vs. 5, = 0.03).
CONCLUSIONS
Our findings support a unique mutational landscape of urethral melanoma compared to cutaneous melanoma. Survival remains poor and is unchanged over the time studied.
PubMed: 36277327
DOI: 10.3233/BLC-211633 -
Gynecologic Oncology Dec 2022Gynecologic tract melanoma (GTM) is a rare malignancy with historically poor outcomes. The current study examines patterns of care and oncologic outcomes in a large...
BACKGROUND
Gynecologic tract melanoma (GTM) is a rare malignancy with historically poor outcomes. The current study examines patterns of care and oncologic outcomes in a large single-institution cohort from the contemporary therapeutic era.
METHODS
Patterns of care and predictors of outcomes were evaluated for all GTM patients without metastatic disease at diagnosis who were treated at our institution between 2009 and 2020 with >6 months of follow-up.
RESULTS
Of the 124 patients included, anatomic subsites were vulvar (n = 82, 66%), vaginal (n = 34, 27%), or cervical (n = 8, 6%). Primary tumor was resected for 85% (n = 106) with surgical nodal evaluation for 60% (n = 75). Systemic therapy, most commonly immune checkpoint inhibitors (ICI, 58% systemic therapy), was used to treat all except one unresectable patient (17/18) and 33% (35/106) of resectable patients. Seven patients received neoadjuvant ICI. Fourteen patients received adjuvant radiation therapy to the pelvis (RT, 13% of those undergoing resection). With a median follow-up of 45 months, 100 patients (81%) recurred. Four-year actuarial outcomes were: 46% local control, 53% nodal control, 36% distant metastasis-free survival, 17% disease-free survival, 49% melanoma-specific survival and 48% overall survival. Mitotic rate > 10/mm, nodal involvement and non-vulvar anatomic subsite were associated with poor outcomes. Patients treated after 2016 did not have significantly better outcomes than those treated earlier.
CONCLUSIONS
Patients with GTM continue to have poor outcomes in the contemporary therapeutic era with particularly notable poor local disease control relative to other mucosal melanoma subtypes. More effective oncologic therapy is needed.
Topics: Humans; Female; Neoplasm Recurrence, Local; Melanoma; Disease-Free Survival; Progression-Free Survival; Disease Progression; Retrospective Studies
PubMed: 36229264
DOI: 10.1016/j.ygyno.2022.09.026 -
Annals of Medicine and Surgery (2012) Sep 2022Vulvar melanoma is a rare malignant tumor of the female genital sphere, representing postmenopausal women's prerogative, the diagnosis is based on immunohistochemicals...
Vulvar melanoma is a rare malignant tumor of the female genital sphere, representing postmenopausal women's prerogative, the diagnosis is based on immunohistochemicals analysis, and treatment requires a multidisciplinary approach. On account of its high metastatic potential as well as the late diagnosis given that it has non-specific clinical signs, the prognosis remains poor. In this study, we report the case of a woman of childbearing who presented a vaginal mass associated to chronic pelvic pain. Paraclinical investigations revealed a right vulvar tumoral process with pathological-looking inguinal adenomegalies on the right side with a necrotic center measuring 16.7 mm on the short axis, micronodules and secondary pulmonary nodules. The patient has been put under palliative chemotherapy, then passed out 8 months later. By this work, we attempt to review the diagnostic circumstances to better understand this delay, also to encourage self-examination and self-screening of abnormal lesions, as well as leveling the awareness of health professionals on this rare disease.
PubMed: 36147087
DOI: 10.1016/j.amsu.2022.104473 -
JID Innovations : Skin Science From... Sep 2022Cases of vulvar melanocytic lesions in juveniles are rarely reported. We analyze the evidence regarding vulvar melanocytic lesions in juveniles with or without vulvar...
Cases of vulvar melanocytic lesions in juveniles are rarely reported. We analyze the evidence regarding vulvar melanocytic lesions in juveniles with or without vulvar lichen sclerosus to help decision making by clinicians and pathologists. A scoping review on vulvar melanocytic lesions with or without vulvar lichen sclerosus, including malignant vulvar melanomas, in females up to age 18 years was performed. In addition, the histopathology records of the cohort of all such lesions in The Netherlands from 1991 through 2020 were investigated, and a structured analysis of tissue samples of the subset of cases with lichen sclerosus was performed. The literature study performed confirms that vulvar melanomas in juveniles are extremely rare and that published case reports are often disputed. In The Netherlands, there are no cases of malignant vulvar melanomas up to age 18 years recorded from 1991 through 2020. Atypical histopathological features are often found in biopsies of vulvar nevi in juveniles, especially with concomitant lichen sclerosus, confirming earlier case studies in the literature. We conclude that even with atypical findings, vulvar melanocytic lesions in juveniles have a benign course. To avoid unnecessary and possibly mutilating procedures, we advise referral to an expert center and adaption of existing guidelines for vulvar melanocytic lesions in juveniles.
PubMed: 36105669
DOI: 10.1016/j.xjidi.2022.100140 -
Medicine Sep 2022Vulvar melanoma is a rare and aggressive tumor with a high risk of local recurrence and distant metastasis. The prognosis is poor with a 5-year overall survival rate of... (Review)
Review
RATIONALE
Vulvar melanoma is a rare and aggressive tumor with a high risk of local recurrence and distant metastasis. The prognosis is poor with a 5-year overall survival rate of only 46.6%. Management of vulvar melanoma remains a clinical challenge. Recent evidences have shown that immune checkpoint inhibitors are effective in the treatment of vulvar melanoma.
PATIENT CONCERNS AND DIAGNOSES
A 63-year-old woman with vulvar malignant melanoma suffered inguinal lymph node metastasis after vulvectomy and chemotherapy. She underwent inguinal lymph node dissection and inguinal radiotherapy. The tumor progressed again and she received immunotherapy.
INTERVENTIONS
The tumor progressed again, and she was admitted to our hospital and received toripalimab combined with apatinib and abraxane.
OUTCOMES
After 6 cycles of immunotherapy, the efficacy achieved partial remission. And with toripalimab as maintenance therapy, the patient achieved durable antitumor efficacy and good safety.
LESSONS
In this rare case, the patient with metastatic vulvar malignant melanoma had durable antitumor efficacy and good safety when receiving toripalimab.
Topics: Antibodies, Monoclonal, Humanized; Female; Humans; Melanoma; Middle Aged; Neoplasms, Second Primary; Skin Neoplasms; Vulvar Neoplasms
PubMed: 36086787
DOI: 10.1097/MD.0000000000030239