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Alveolar soft part sarcoma of the uterine cervix: a case report and review of the literature.

Lee HJ - Korean J Pathol (2014)

Bottom Line: Herein, we describe a 17-yearold female patient who presented with active vaginal bleeding.Immunohistochemically, tumor cells were strongly nuclear positive for transcription factor E3.The patient remained disease free for 24 months without adjuvant therapy.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, Soonchunhyang University Cheonan Hospital, Soonchunhyang University College of Medicine, Cheonan, Korea.

ABSTRACT
Alveolar soft part sarcoma (ASPS) of the uterine cervix is a rare malignancy, and 21 cases have been reported the literature from every language (including our case). Herein, we describe a 17-yearold female patient who presented with active vaginal bleeding. Pelvic examination revealed a 1.6 ×1.0×0.5-cm-sized soft mass protruding from the uterine cervix. The final pathological diagnosis was ASPS of the uterine cervix. Immunohistochemically, tumor cells were strongly nuclear positive for transcription factor E3. The patient remained disease free for 24 months without adjuvant therapy. The prognosis of ASPS in the cervix is considerably better than that of ASPS in soft tissues due to early clinical detection, small size, and resectability. ASPS should be considered in the differential diagnosis of an unusual epithelioid neoplasm showing organoid appearance with mild cytologic atypia and no/rare mitotic figures, particularly in young women. Pathologists should be aware of those unusual locations where ASPS may originate.

No MeSH data available.


Related in: MedlinePlus

Representative histologic features of an alveolar soft part sarcoma. (A) Solid nest arrangement with stromal hyalinization. (B) Groups of tumor cells are outlined by delicate connective tissue septa. The tumor cells show prominent nucleoli and abundant eosinophilic cytoplasm. No mitotic activity is found.
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f2-kjpathol-48-5-361: Representative histologic features of an alveolar soft part sarcoma. (A) Solid nest arrangement with stromal hyalinization. (B) Groups of tumor cells are outlined by delicate connective tissue septa. The tumor cells show prominent nucleoli and abundant eosinophilic cytoplasm. No mitotic activity is found.

Mentions: We describe a 17-year-old female patient, gravida 0, who presented with active vaginal bleeding for three days with severe anemia after coitus. Her past medical and familial histories were unremarkable. Pelvic examination revealed a protruding 1.6× 1.0×0.5-cm-sized, soft mass in the lateral wall of the uterine cervix. Computed tomography examination revealed a mass lesion, 1.6 cm in diameter, mainly located in the vagina originating in the uterine cervix (Fig. 1) and no evidence of distant metastasis. The initial clinical diagnosis was a cervical myoma. Cervical cytology was negative and the results of routine laboratory studies were unremarkable, except for a decreased hemoglobin and hematocrit level. The hemoglobin level was 5.1 g/dL (normal range, 12 to 16 g/dL), and the hematocrit level was 16.4% (normal range, 37% to 47%). Tumor markers cancer antigen (CA) 125, CA19-9, and carcinoembryonic antigen were all within normal limits. A transvaginal cervical myomectomy was carried out. During the procedure, the cervical mass was fragile and hemorrhage was encountered. The surgically-resected tumor was well-circumscribed and located beneath the surface mucosa. The tumor cells were relatively uniform in appearance and exhibited a solid nested architectural pattern (Fig. 2A). The nests of cells were separated by thicker fibrous septa and stromal hyalinization. The tumor was composed of large polygonal cells with distinct cell borders and abundant eosinophilic cytoplasm. The round to oval nuclei often contained single prominent nucleoli (Fig. 2B). The chromatin was uniformly arranged and the nuclear pleomorphism was mild to moderate. Neither mitotic figures nor necrosis were observed. The nucleus/cytoplasm (N/C) ratio was not high due to abundant eosinophilic cytoplasm. No vascular invasion was present. Periodic acid–Schiff (PAS) and PAS with diastase staining showed few granules and no rod-shaped crystals (Fig. 3A, B). Immunohistochemically, the tumor cells stained diffusely nuclear positive for TFE3 (Fig. 3C), positive for MyoD1 (Fig. 3D), and focal positive for smooth muscle actin (SMA), neuron specific enolase (NSE), and myoglobin. The Ki-67 proliferating index was 3%. In addition, ASPS was negative for epithelial markers (cytokeratin [CK], high-molecular weight cytokeratin [HMWCK], CK8/18), neuroendocrine markers (CD56, chromogranin A, synaptophysin), melanocytic markers (human melanoma black 45 [HMB45], S-100), myogenic markers (desmin, myogenin), germ cell tumor markers (alpha-feto protein [AFP], CD30, human chorionic gonadotropin [HCG], placental-like alkaline phosphatase [PLAP], CD10), vimentin, and CD34. The final pathological diagnosis was ASPS of the uterine cervix.


Alveolar soft part sarcoma of the uterine cervix: a case report and review of the literature.

Lee HJ - Korean J Pathol (2014)

Representative histologic features of an alveolar soft part sarcoma. (A) Solid nest arrangement with stromal hyalinization. (B) Groups of tumor cells are outlined by delicate connective tissue septa. The tumor cells show prominent nucleoli and abundant eosinophilic cytoplasm. No mitotic activity is found.
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4215961&req=5

f2-kjpathol-48-5-361: Representative histologic features of an alveolar soft part sarcoma. (A) Solid nest arrangement with stromal hyalinization. (B) Groups of tumor cells are outlined by delicate connective tissue septa. The tumor cells show prominent nucleoli and abundant eosinophilic cytoplasm. No mitotic activity is found.
Mentions: We describe a 17-year-old female patient, gravida 0, who presented with active vaginal bleeding for three days with severe anemia after coitus. Her past medical and familial histories were unremarkable. Pelvic examination revealed a protruding 1.6× 1.0×0.5-cm-sized, soft mass in the lateral wall of the uterine cervix. Computed tomography examination revealed a mass lesion, 1.6 cm in diameter, mainly located in the vagina originating in the uterine cervix (Fig. 1) and no evidence of distant metastasis. The initial clinical diagnosis was a cervical myoma. Cervical cytology was negative and the results of routine laboratory studies were unremarkable, except for a decreased hemoglobin and hematocrit level. The hemoglobin level was 5.1 g/dL (normal range, 12 to 16 g/dL), and the hematocrit level was 16.4% (normal range, 37% to 47%). Tumor markers cancer antigen (CA) 125, CA19-9, and carcinoembryonic antigen were all within normal limits. A transvaginal cervical myomectomy was carried out. During the procedure, the cervical mass was fragile and hemorrhage was encountered. The surgically-resected tumor was well-circumscribed and located beneath the surface mucosa. The tumor cells were relatively uniform in appearance and exhibited a solid nested architectural pattern (Fig. 2A). The nests of cells were separated by thicker fibrous septa and stromal hyalinization. The tumor was composed of large polygonal cells with distinct cell borders and abundant eosinophilic cytoplasm. The round to oval nuclei often contained single prominent nucleoli (Fig. 2B). The chromatin was uniformly arranged and the nuclear pleomorphism was mild to moderate. Neither mitotic figures nor necrosis were observed. The nucleus/cytoplasm (N/C) ratio was not high due to abundant eosinophilic cytoplasm. No vascular invasion was present. Periodic acid–Schiff (PAS) and PAS with diastase staining showed few granules and no rod-shaped crystals (Fig. 3A, B). Immunohistochemically, the tumor cells stained diffusely nuclear positive for TFE3 (Fig. 3C), positive for MyoD1 (Fig. 3D), and focal positive for smooth muscle actin (SMA), neuron specific enolase (NSE), and myoglobin. The Ki-67 proliferating index was 3%. In addition, ASPS was negative for epithelial markers (cytokeratin [CK], high-molecular weight cytokeratin [HMWCK], CK8/18), neuroendocrine markers (CD56, chromogranin A, synaptophysin), melanocytic markers (human melanoma black 45 [HMB45], S-100), myogenic markers (desmin, myogenin), germ cell tumor markers (alpha-feto protein [AFP], CD30, human chorionic gonadotropin [HCG], placental-like alkaline phosphatase [PLAP], CD10), vimentin, and CD34. The final pathological diagnosis was ASPS of the uterine cervix.

Bottom Line: Herein, we describe a 17-yearold female patient who presented with active vaginal bleeding.Immunohistochemically, tumor cells were strongly nuclear positive for transcription factor E3.The patient remained disease free for 24 months without adjuvant therapy.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, Soonchunhyang University Cheonan Hospital, Soonchunhyang University College of Medicine, Cheonan, Korea.

ABSTRACT
Alveolar soft part sarcoma (ASPS) of the uterine cervix is a rare malignancy, and 21 cases have been reported the literature from every language (including our case). Herein, we describe a 17-yearold female patient who presented with active vaginal bleeding. Pelvic examination revealed a 1.6 ×1.0×0.5-cm-sized soft mass protruding from the uterine cervix. The final pathological diagnosis was ASPS of the uterine cervix. Immunohistochemically, tumor cells were strongly nuclear positive for transcription factor E3. The patient remained disease free for 24 months without adjuvant therapy. The prognosis of ASPS in the cervix is considerably better than that of ASPS in soft tissues due to early clinical detection, small size, and resectability. ASPS should be considered in the differential diagnosis of an unusual epithelioid neoplasm showing organoid appearance with mild cytologic atypia and no/rare mitotic figures, particularly in young women. Pathologists should be aware of those unusual locations where ASPS may originate.

No MeSH data available.


Related in: MedlinePlus