case_images: PMC9459382_01
case_images: The tumour was found to be located below the right labia majora, and its surface was covered with massive, engorged blood vessels.
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case_images: f1
case_images: Upon gynaecologic examination, an immobile, solid, and involved mass with no bleeding was found on the inferior margin of the right labia majora and the lateral margin of the hymen ( Figure 1 ). During the operation, the tumour was found to be located below the right labia majora, and its surface was covered with massive, engorged blood vessels ( Figure 1 ).
case_images: (Primary Treatment) Magnetic resonance imaging (MRI) scans: A mass on the right vulva measuring 6.0 cm*5.5 cm*4.3 cm in size, involving the right levator ani muscle, was observed. The tumour invaded the vaginal wall and disrupted the continuity of its mucous. (A) T1 weighted imaging (T1WI); (B) Diffusion Weighted Imaging (DWI); (C) Enhanced; (D) Enhanced.
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case_images: f2
case_images: In addition, the tumour invaded the vaginal wall and disrupted the continuity of its mucous ( Figure 2 ).
case_images: (Recurrence Treatment) Pelvic MRI showing a mass measuring 4.3 cm*3.6 cm*5.4 cm in size, suggesting that the lesion was closely related to the right posterior vaginal wall and anterior rectal wall. (A) T1 weighted imaging (T1WI); (B) T2 weighted imaging (T2WI); (C) Diffusion Weighted Imaging (DWI); (D) T2 weighted imaging (T2WI).
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case_images: f3
case_images: Whole-body CT examination was performed to exclude primary tumours in other sites, and pelvic scan plus enhanced MRI ( Figure 3 ) and transanal ultrasound were used to clarify the extent of tumour invasion.
case_images: (A) The mass was dissected under the table and was approximately 6*4*4 cm in size, (B)The mass was hard and have a greyish white myxoma-like cut surface.
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case_images: f4
case_images: At this time, the resected mass was approximately 6*4*4 cm in size, hard and have a greyish white myxoma-like cut surface ( Figure 4 ).
case_images: (Primary Treatment) Immunohistochemical staining: (A) The tumour cells were composed of chief cells and sustentacular cells, and they were clustered in small nests, here called zellballen, demarcated by delicate fibrous stroma and capillaries. (B) Immunohistochemical staining CgA(+); (C) Immunohistochemical staining SYN(+); (D) Immunohistochemical staining S-100(-).
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case_images: PMC9459382_01_fonc-12-961666-g005.jpg
case_images: f5
case_images: The immunohistochemistry results at the time of primary treatment were as follows: the tumour was found to be positive for Syn, CgA, and Desmin but negative for protein S-100, human melanoma-associated antigen, MelanA, CK, CD10, CD68, CD31, and PAX-8 ( Figure 5 ).
case_images: (Recurrence Treatment)Immunohistochemical staining: (A) Immunohistochemical staining CgA(+); (B) Immunohistochemical staining SYN(+); (C) Immunohistochemical staining S-100(+); (D) Immunohistochemical staining SSTR2 (+).
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case_images: f6
case_images: The immunohistochemistry results at the time of recurrence treatment were as follows: CgA (+), CD56 (+), Syn (+), somatostatin receptor (SSTR2) (+), S-100 (+), SOX-10 (individual cells +), CK (-), and P53 (-), with a Ki-67 positive rate of approximately 2-3% ( Figure 6 ).