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Journal of Cancer Research and... 2022Malignant melanoma of the anorectal region is a very rare aggressive malignant neoplasm and it constitutes 1% of all malignant lesions of this area. About 70% of these...
Malignant melanoma of the anorectal region is a very rare aggressive malignant neoplasm and it constitutes 1% of all malignant lesions of this area. About 70% of these lesions are pigmented, whereas 30% are amelanotic. Demonstration of immune markers of melanoma by immunohistochemistry (IHC) is required for confirming the diagnosis of amelanotic malignant melanoma. Here, we report a case of anorectal malignant amelanotic melanoma in a 65-year-old male with no medical comorbidities, who presented with chief complaints of bleeding per rectum associated with prolapsing mass per rectum of 7 months duration. On external examination and proctoscopy, three prolapsed pedunculated fungating masses were seen externally protruding out of the rectum approximately 4 cm from the anal verge. Contrast-enhanced computed tomography of the whole abdomen and pelvis was suggestive of moderately enhancing lobulated anorectal mass with large polypoidal intraluminal component arising from anorectal walls and extension into mid-lower rectum with liver and locoregional lymph nodes metastasis. The patient was taken up for palliative local excision. Per-operatively, three large irregular highly vascular pedunculated rectal growth was seen. The growth was excised and sent for histopathological examination. Microscopic examination of mass show spindle-to-ovoid tumor cells with hyperchromatic central to eccentric nuclei arranged in intersecting fascicles with a focal alveolar pattern. The large number of atypical mitotic figures (40-50/10 High Power Field (HPF)) was seen along with areas of necrosis and the presence of few bizarre binucleated and multinucleated giant cells. A differential diagnosis of malignant amelanotic melanoma was given along with undifferentiated carcinoma, gastrointestinal stromal tumor , and Non-Hodgkin's lymphoma. On IHC, the tumor cells were reactive for HMB45, S-100, and SOX-10. Thus a diagnosis of malignant amelanotic melanoma was confirmed. The patient had symptomatic improvement.
Topics: Aged; Gastrointestinal Stromal Tumors; Humans; Immunohistochemistry; Male; Melanoma, Amelanotic; Rectal Neoplasms; Skin Neoplasms
PubMed: 35381792
DOI: 10.4103/jcrt.JCRT_461_20 -
Frontiers in Oncology 2022To describe the outcome of intraocular tumor resection by partial transscleral sclerouvectomy (PTSU) combined with micro-invasive vitrectomy and reconstruction of the...
Surgical Resection of Intraocular Tumors (Partial Transscleral Sclerouvectomy Combined With Mircoinvasive Vitrectomy and Reconstruction of the Eyeball) in Asian Patients: Twenty-Five Years Results.
OBJECTIVE
To describe the outcome of intraocular tumor resection by partial transscleral sclerouvectomy (PTSU) combined with micro-invasive vitrectomy and reconstruction of the eyeball (MVRE) in Asian patients.
DESIGN METHODS AND PARTICIPANTS
This retrospective, interventional cohort study included 366 patients who underwent PTSU combined with MVRE for intraocular tumors both in adult and pediatric age groups. The medical records of these patients were reviewed for clinical, operative, and histopathological features.
MAIN OUTCOME MEASURES
Globe salvage, best corrected visual acuity (BCVA), surgical side effects, tumor control, and tumor-related metastasis and death.
RESULTS
The mean follow-up duration was 87 months (median, 66; range, 1-303 months). Among the 366 patients, the mean age was 8.5 years (median, 7; range, 1-19 years) in the 37 pediatric patients, and was 43 years (median, 42; range, 20-51) in 329 adult patients. The tumor mainly involved the ciliary body (n=136; 37.2%) and choroid (n=86; 23.5%). The common pathologic diagnosis of the 366 patients was as follows. In the pediatric age group, histopathologic examination revealed positive tumor margins in 37 patients mainly including ciliary body medulloepithelioma (8/37), ciliary body melanocytoma (13/37) and uveal melanoma (5/37). In the adult group, the pathological diagnosis mainly included melanoma (195/329), RPE adenoma (21/329), amelanotic melanoma (13/329), ciliary body adenoma of nonpigmented epithelium (19/329), schwannoma/neurilemmoma (11/329), melanocytoma (24/329), and leiomyoma (9/329). The globe salvage rate was 81.1% in the pediatric age groups (<20 years), and 93.6% in the adult group (≥20 years), respectively. Of the 338 salvaged eyes, final BCVA was 20/20 to 20/40 in 16 (4.7%), 20/40 to 20/80 in 58 (17.2%), 20/80 to 20/200 in 160 (47.3%), and ≤ 20/200 in 104 (30.8%). Early side effects included corneal edema in 28 (7.7%) patients, hyphema in 46 (12.6%), and vitreous hemorrhage in 76 (21%) patients. Postoperative side effects included proliferative vitreoretinopathy (PVR) in 67 (18.3%), late cataract in 42 (11.5%), and glaucoma in 18 (5%) patients. Local tumor recurrence was detected in 20 patients (5.5%) at a mean interval of 23.6 months, including melanoma (n=19) and medulloepithelioma (n=1). Enucleation was necessary in 28 (7.7%) cases owing to recurrence in 15 (53.6%), eye prophylaxis with high-grade malignancy in 5 (17.8%), and blind painful eye in 8 (28.6%) cases. Kaplan-Meier estimated for 5, 10-year metastasis rate and metastasis-related death rate (95%CI) in 213 UM patients were 3.2% (1.4%-7.0%), 6.9% (3.8%-12.3%); and 3.5% (1.6%-7.6%), 7.6% (4.2%-13.5%), respectively.
CONCLUSIONS
As a surgically challenging procedure, PTSU combined with MVRE offers several theoretical advantages over enucleation and radiotherapy. It can achieve control of most intraocular tumors, preserve useful vision, and maintain a cosmetically normal eye.
PubMed: 35371976
DOI: 10.3389/fonc.2022.768635 -
Cureus Feb 2022Sinonasal malignant melanoma is a rare but aggressive tumor of the head and neck area. It has a poor prognosis. Common symptoms are nasal obstruction, epistaxis, or...
Sinonasal malignant melanoma is a rare but aggressive tumor of the head and neck area. It has a poor prognosis. Common symptoms are nasal obstruction, epistaxis, or purulent rhinorrhea. Diagnosis relies on histopathology with immunohistochemistry (IHC) studies. Surgery is the essential treatment, most often supplemented by radiotherapy or immunotherapy. A 63-year-old female patient, with a history of right dacryocystorhinostomy and Parkinson's disease, consulted for symptoms of right nasal obstruction with increasing intensity accompanied by two episodes of mild unilateral epistaxis. Rigid optic examination showed a white-pinkish right obstructive supra-centimetric endonasal tumor. CT revealed an extensive tissue process of the right nasal cavity invading the maxillary sinus, the inferior and middle conchas. A biopsy of the lesion was conducted under local anesthesia. The immunohistochemical study has shown undifferentiated tumor with positive antibody anti PS100 and anti-melan A evoking malignant sinonasal melanoma. The patient underwent two surgeries for maxillectomies as she presented a first local recurrence. She was started on adjuvant radiotherapy. At one year of follow-up, she does not present any local or general signs of disease. Sinonasal melanoma is a particular entity of head and neck mucosal melanomas. The highest incidence is described to be in the seventh and eighth decades of life with no sex difference. IHC profiling of different melanoma subtypes showed the importance of alterations in the KIT gene, this genetic data may constitute a therapeutic target. After surgery, the important local recurrence rates and regional failure justify adjuvant radiotherapy also for resections in free margins. Most authors consider that prophylactic neck dissection is not necessary. Preoperative imaging features (CT scan) are characteristic and helpful for diagnosis. IHC is essential, has a high sensitivity for differentiating achromic melanomas from other neoplasms. Sinonasal achromic melanoma is a very uncommon tumor, invasive, and frequently associated with distant metastasis. Paraclinic examinations are essential for staging and guiding therapeutic management. Immunotherapy is a promising ground of research as it comes to metastatic and advanced disease.
PubMed: 35371814
DOI: 10.7759/cureus.22442 -
BMJ Case Reports Mar 2022Primary amelanotic melanoma is an infrequent occurrence in the oral cavity. Owing to the high rate of local invasion and distant metastasis, oral amelanotic melanoma...
Primary amelanotic melanoma is an infrequent occurrence in the oral cavity. Owing to the high rate of local invasion and distant metastasis, oral amelanotic melanoma (OAM) carries a very poor prognosis. The absence of pathognomonic clinical and routine histological features in OAM is the reason for diagnosticdelay, which further worsens the prognosis. This case report discusses the masquerading nature of OAM that was clinically and histologically mimicking several malignant neoplasms. This case also demonstrates the poor prognosis of OAM. The objective of presenting this case is that the diagnostic delay of OAM can be avoided through enhanced clinical awareness and subsequent appropriate immunohistochemical investigations, in addition to the routine H&E-stained histopathological evaluation.
Topics: Delayed Diagnosis; Humans; Melanoma, Amelanotic; Mouth; Palate; Skin Neoplasms
PubMed: 35354566
DOI: 10.1136/bcr-2022-249027 -
BMC Gastroenterology Mar 2022Primary malignant melanoma of esophagus (PMME) is an extremely rare disease with poor prognosis. We aimed to determine the clinical characteristics and treatment...
BACKGROUND
Primary malignant melanoma of esophagus (PMME) is an extremely rare disease with poor prognosis. We aimed to determine the clinical characteristics and treatment outcomes of patients with PMME.
METHODS
We retrospectively reviewed 17 patients diagnosed with PMME in Samsung Medical Center between 2000 and 2020 with median 34 months of follow-up. Survival outcomes were analyzed with Kaplan-Meier method.
RESULTS
15 patients (88.2%) were male and the most common presenting symptom was dysphagia (9/17, 52.9%). On endoscopy, tumors were mass-forming in 15 patients (88.2%) and diffusely infiltrative in two patients (11.8%). Lesions were melanotic in 13 patients (76.5%) and amelanotic in four patients (23.5%). The most common tumor location was lower esophagus (11/17, 64.7%). The disease was metastatic at the time of diagnosis in four patients (23.5%). As for treatment, 10 patients (58.8%) underwent surgery. In all 17 patients, the median overall survival was 10 months. In surgically treated patients, all patients experienced recurrence and the median disease-free survival was 4 months. There was no statistical difference in overall survival between patients with or without surgery. Patients with diffusely infiltrative tumor morphology had better overall survival compared to those with mass-forming tumor morphology (P = 0.048). Two patients who received immunotherapy as the first-line treatment without surgery showed overall survival of 34 and 18 months, respectively.
CONCLUSIONS
As radical resection for patients with PMME does not guarantee favorable treatment outcomes, novel treatment strategy is required. Further large-scale studies are warranted to determine the efficacy of immunotherapy for patients with PMME.
Topics: Esophagus; Humans; Male; Melanoma; Retrospective Studies; Skin Neoplasms; Treatment Outcome
PubMed: 35351022
DOI: 10.1186/s12876-022-02235-8 -
Italian Journal of Dermatology and... Oct 2022Nevoid melanoma (NeM) is a rare variant of melanoma resembling melanocytic nevus. The aim of the study was to systematically review the dermoscopic features of NeM.
BACKGROUND
Nevoid melanoma (NeM) is a rare variant of melanoma resembling melanocytic nevus. The aim of the study was to systematically review the dermoscopic features of NeM.
METHODS
A hospital-based retrospective study was conducted. Dermoscopic features of NeMs diagnosed through excisional biopsy between January 2015 1, and March 1, 2021, were compared to superficial spreading melanomas (SSMs) matched by Breslow's thickness. Then, a literature search was performed. Electronic searches on PubMed database via Medline were conducted to retrieve any manuscript reporting detailed dermoscopic features of histopathologically confirmed NeM.
RESULTS
A total of 60 malignant melanomas (MM) comprising 20 NeM and 40 SSM were collected. Twelve out of 20 (60%) NeM showed a nevus-like appearance, including reticular and globular patterns, and in 35% of these cases it was detected because of dermoscopic changes. Then, a total of seven original manuscripts were retrieved from the literature review, comprising 56 cases overall. NeM showed nevus-like pattern in 53% of the cases, multicomponent pattern in 21% and amelanotic in 9%. Enlargement, irregularly distributed dots/globules, irregular pigmentation, and atypical vascular pattern were found in NeM with nevus-like appearance. NeM with multicomponent pattern were characterized by irregular pigmentation, blue-white veil, irregular dots and atypical vascular pattern. Amelanotic NeM is rare and show atypical vascular pattern and milia-like cysts.
CONCLUSIONS
Dermoscopy of NeM is challenging as it frequently shows a nevus-like pattern, but clues and detection of dermoscopic changes may help to identify it.
Topics: Dermoscopy; Epidermal Cyst; Humans; Melanoma; Melanoma, Amelanotic; Nevus; Nevus, Pigmented; Retrospective Studies; Skin Neoplasms; Melanoma, Cutaneous Malignant
PubMed: 35274882
DOI: 10.23736/S2784-8671.22.07262-0 -
Actas Dermo-sifiliograficas Jan 2022Desmoplastic melanoma (DM) accounts for 0.4% to 4% of all melanomas. These skin tumors are mainly formed by amelanotic spindled melanocytes immersed in an abundant... (Review)
Review
Desmoplastic melanoma (DM) accounts for 0.4% to 4% of all melanomas. These skin tumors are mainly formed by amelanotic spindled melanocytes immersed in an abundant collagen stroma and are classified as pure when the desmoplastic component accounts for at least 90% of the invasive tumor and as mixed or combined otherwise. DMs are more common in men (male to female ratio, 1.7 to 2:1), and the mean age at diagnosis is 66 to 69 years. The tumors tend to occur in chronically sun-exposed areas, often in association with lentigo maligna, and are difficult to recognize because they can resemble a scar, presenting as a firm, unpigmented papule or plaque with poorly defined borders. DMs also have a strong tendency to recur locally, and pure variants rarely spread to the lymph nodes. Nonetheless, recently published series suggest that patients with DM have a similar prognosis to those with nondesmoplastic melanoma of the same thickness. The clinical management of DM varies in certain aspects from that of other melanomas and is reviewed in this article.
PubMed: 35249710
DOI: 10.1016/j.ad.2021.06.004 -
BMJ Case Reports Mar 2022Ocular surface melanoma (OSM) is rare. An 81-year-old Caucasian woman presented with a 4-month history of right eye pain and reduced vision. Histopathological...
Ocular surface melanoma (OSM) is rare. An 81-year-old Caucasian woman presented with a 4-month history of right eye pain and reduced vision. Histopathological examination of the excisional biopsy identified invasive amelanotic melanoma of the conjunctiva expressing Melan A and SOX10. X-ray of chest, CT of liver and MRI of the brain and orbit did not identify macroscopic metastases. She was given adjuvant topical mitomycin-C 0.04% for four cycles of 2 weeks. Her vision improved and the cornea was clear at 6 months.
Topics: Aged, 80 and over; Eye Neoplasms; Female; Humans; Melanoma; Mitomycin; Orbit; Skin Neoplasms
PubMed: 35246440
DOI: 10.1136/bcr-2021-248725 -
Retina (Philadelphia, Pa.) Apr 2022To report the clinicopathologic correlation of a case of bilateral serpiginous-like chorioretinitis (SLC) associated with unilateral ciliochoroidal melanoma.
PURPOSE
To report the clinicopathologic correlation of a case of bilateral serpiginous-like chorioretinitis (SLC) associated with unilateral ciliochoroidal melanoma.
METHODS
A 71-year-old white woman was diagnosed with progressive SLC in both eyes associated with ciliochoroidal melanoma in the right eye. Clinical findings and imaging before and after enucleation in the right eye were correlated to histologic and immunohistochemistry sections.
RESULTS
Examination and imaging identified a peripheral bilobed amelanotic lesion with low reflectivity on B-scan ultrasound with an associated exudative detachment in the right eye. Additionally, multiple areas of new SLC lesions in the macula and peripapillary region in the right eye and along the inferior arcade in the left eye were observed. Oncologic evaluation confirmed a Class 2, ciliochoroidal melanoma, and the eye was enucleated. Autoimmune and infectious laboratory evaluations for the etiology of the SLC lesions were negative. Histopathology of the enucleated eye confirmed the diagnosis of uveal melanoma with lymphocytic inflammation at the edges of the tumor itself and in the areas of discrete SLC lesions. Immunohistochemistry identified similar predominantly CD3 and CD8 T cells and fewer CD20 B cells in both regions.
CONCLUSION
Serpiginous-like chorioretinitis may present as a paraneoplastic, predominantly T-lymphocyte inflammation associated with intraocular tumor such as uveal melanoma.
Topics: Aged; Chorioretinitis; Female; Humans; Inflammation; Melanoma; Uveal Neoplasms
PubMed: 35174798
DOI: 10.1097/IAE.0000000000003435 -
Annals of Medicine and Surgery (2012) Feb 2022and importance: Amelanotic melanoma is a rare and aggressive type of melanoma. It is often diagnosed late because of the lack of melanin in its cells, and this causes...
INTRODUCTION
and importance: Amelanotic melanoma is a rare and aggressive type of melanoma. It is often diagnosed late because of the lack of melanin in its cells, and this causes treatment delay and, eventually, poor prognosis.
CASE PRESENTATION
We report a case of a 79-year-old female patient that presented to the dermatology clinic with an asymptomatic lesion on the medial heel of the right foot, with no medical history of previous melanoma or related skin cancer. To get the right diagnosis, an incisional biopsy was performed, and the sample was sent to the pathology laboratory. The sample was stained with S100 and HMB-45 stains, and both were positive. Also, no melanin pigmented cells were seen, so the diagnosis was amelanotic nodular melanoma. The patient was then referred to surgery. The lesion was successfully excised with 5cm safety margins, and the whole lesion was sent to the pathology laboratory to ensure that the edges are malignancy-free. After 18 months of follow-up, the patient is in good health.
CONCLUSION
Accurate and early diagnosis with appropriate clinical intervention can improve the prognosis and reduce mortality and morbidity rates.
PubMed: 35145679
DOI: 10.1016/j.amsu.2022.103316