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Large cell neuroendocrine carcinoma originating from the uterine endometrium: a report on magnetic resonance features of 2 cases with very rare and aggressive tumor.

Makihara N, Maeda T, Nishimura M, Deguchi M, Sonoyama A, Nakabayashi K, Kawakami F, Itoh T, Yamada H - Rare Tumors (2012)

Bottom Line: Neuroendocrine carcinomas (NEC) of the female genital tract are aggressive and uncommon tumors, which usually involve the uterine cervix and ovary, and are seen very rarely in the endometrium.Only less than 10 cases of large cell NEC (LCNEC) of the endometrium have been reported in the literature and their radiological findings are not well described.In both cases, the uterine body was enlarged and the tumor occupied part of the uterine cavity.

View Article: PubMed Central - PubMed

Affiliation: Department of Obstetrics and Gynecology.

ABSTRACT
Neuroendocrine carcinomas (NEC) of the female genital tract are aggressive and uncommon tumors, which usually involve the uterine cervix and ovary, and are seen very rarely in the endometrium. Only less than 10 cases of large cell NEC (LCNEC) of the endometrium have been reported in the literature and their radiological findings are not well described. We report here two cases of pathologically proven LCNEC of the uterine endometrium. In both cases, the uterine body was enlarged and the tumor occupied part of the uterine cavity. Endometrial mass exhibited heterogeneous high intensity on T2-weighted magnetic resonance (MR) images, and diffusion-weighted MR images revealed high intensity throughout the tumor, consistent with malignancy. LCNEC is a highly malignant neoplasm without particular findings in terms of diagnostic imaging and pathology, so its preoperative definitive diagnosis is very difficult. However, when laboratory test, pathologic diagnosis and MR imaging suggest a poorly differentiated uterine malignancy, positron emission tomography-computed tomography scan should be performed as a general assessment to help with diagnosis.

No MeSH data available.


Related in: MedlinePlus

A) T2 weighted image shows the mass with heterogeneous high intensity (arrow); B) contrast enhanced T1 weighted image shows heterogeneous enhancement of the tumor (arrow); C) diffusion-weighted image shows high intensity throughout the tumor (arrow).
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Figure 3: A) T2 weighted image shows the mass with heterogeneous high intensity (arrow); B) contrast enhanced T1 weighted image shows heterogeneous enhancement of the tumor (arrow); C) diffusion-weighted image shows high intensity throughout the tumor (arrow).

Mentions: Physical examination revealed that she had an enlarged uterus. The laboratory tests were unremarkable, except for the elevation of serum lactate dehydrogenase (279 IU/L; reference level, 115∼217 IU/L). The MR images demonstrated a large endometrial mass of heterogeneous high intensity expanding in the endometrial cavity on T2-weighted images (Figure 3A), with part of the mass infiltrating to the uterine serosa throughout the myometrium. The mass revealed hypointensity including high intensity foci, suggestive of intratumoral hemorrhage, on T1-weighted images. Contrast-enhanced T1-weighted MR images showed heterogeneously enhanced tumor (Figure 3B), and diffusion-weighted MR images revealed high intensity throughout the tumor (Figure 3C), consistent with a malignant uterine tumor. Paraaortic lymphadenopathies were identified on the abdominal CT. She underwent abdominal total hysterectomy with bilateral salpingo-oophorectomy, omentectomy, and pelvic-paraaortic lymphadenectomy. Grossly, the uterine cavity was dilated and showed a white tumor involving the fundic region, and the tumor infiltrated to the myometrium deeply. The isthmic and cervical areas were uninvolved, and the bilateral adnexa and omentum were free of metastasis. Microscopically, the carcinoma displays a neuroendocrine morphology, including organoid nesting with large zones of necrosis, trabecular growth, rosettes and peripheral palisading patterns (Figure 4A). Immunohistochemistry revealed diffuse positivity for synaptophysin, chromogranin, CD56 and p53 (Figure 4B). The pathologic diagnosis was appropriate for large cell neuroendocrine carcinoma of the endometrium, and because of the metastasis to right internal iliac lymph nodes and left external iliac lymph node, the final stage was 3c. She was thereafter treated by chemotherapy (intravenous cisplatin; 60 mg/m2 and irinotecan; 60 mg/m2). After 6 cycles of chemotherapy, this patient was disease-free during 6 months. However, FDG-PET/CT, which was performed 13 months after the operation, revealed increased FDG uptake in paraaortic lymph nodes, being suspected of the recurrence.


Large cell neuroendocrine carcinoma originating from the uterine endometrium: a report on magnetic resonance features of 2 cases with very rare and aggressive tumor.

Makihara N, Maeda T, Nishimura M, Deguchi M, Sonoyama A, Nakabayashi K, Kawakami F, Itoh T, Yamada H - Rare Tumors (2012)

A) T2 weighted image shows the mass with heterogeneous high intensity (arrow); B) contrast enhanced T1 weighted image shows heterogeneous enhancement of the tumor (arrow); C) diffusion-weighted image shows high intensity throughout the tumor (arrow).
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC3475944&req=5

Figure 3: A) T2 weighted image shows the mass with heterogeneous high intensity (arrow); B) contrast enhanced T1 weighted image shows heterogeneous enhancement of the tumor (arrow); C) diffusion-weighted image shows high intensity throughout the tumor (arrow).
Mentions: Physical examination revealed that she had an enlarged uterus. The laboratory tests were unremarkable, except for the elevation of serum lactate dehydrogenase (279 IU/L; reference level, 115∼217 IU/L). The MR images demonstrated a large endometrial mass of heterogeneous high intensity expanding in the endometrial cavity on T2-weighted images (Figure 3A), with part of the mass infiltrating to the uterine serosa throughout the myometrium. The mass revealed hypointensity including high intensity foci, suggestive of intratumoral hemorrhage, on T1-weighted images. Contrast-enhanced T1-weighted MR images showed heterogeneously enhanced tumor (Figure 3B), and diffusion-weighted MR images revealed high intensity throughout the tumor (Figure 3C), consistent with a malignant uterine tumor. Paraaortic lymphadenopathies were identified on the abdominal CT. She underwent abdominal total hysterectomy with bilateral salpingo-oophorectomy, omentectomy, and pelvic-paraaortic lymphadenectomy. Grossly, the uterine cavity was dilated and showed a white tumor involving the fundic region, and the tumor infiltrated to the myometrium deeply. The isthmic and cervical areas were uninvolved, and the bilateral adnexa and omentum were free of metastasis. Microscopically, the carcinoma displays a neuroendocrine morphology, including organoid nesting with large zones of necrosis, trabecular growth, rosettes and peripheral palisading patterns (Figure 4A). Immunohistochemistry revealed diffuse positivity for synaptophysin, chromogranin, CD56 and p53 (Figure 4B). The pathologic diagnosis was appropriate for large cell neuroendocrine carcinoma of the endometrium, and because of the metastasis to right internal iliac lymph nodes and left external iliac lymph node, the final stage was 3c. She was thereafter treated by chemotherapy (intravenous cisplatin; 60 mg/m2 and irinotecan; 60 mg/m2). After 6 cycles of chemotherapy, this patient was disease-free during 6 months. However, FDG-PET/CT, which was performed 13 months after the operation, revealed increased FDG uptake in paraaortic lymph nodes, being suspected of the recurrence.

Bottom Line: Neuroendocrine carcinomas (NEC) of the female genital tract are aggressive and uncommon tumors, which usually involve the uterine cervix and ovary, and are seen very rarely in the endometrium.Only less than 10 cases of large cell NEC (LCNEC) of the endometrium have been reported in the literature and their radiological findings are not well described.In both cases, the uterine body was enlarged and the tumor occupied part of the uterine cavity.

View Article: PubMed Central - PubMed

Affiliation: Department of Obstetrics and Gynecology.

ABSTRACT
Neuroendocrine carcinomas (NEC) of the female genital tract are aggressive and uncommon tumors, which usually involve the uterine cervix and ovary, and are seen very rarely in the endometrium. Only less than 10 cases of large cell NEC (LCNEC) of the endometrium have been reported in the literature and their radiological findings are not well described. We report here two cases of pathologically proven LCNEC of the uterine endometrium. In both cases, the uterine body was enlarged and the tumor occupied part of the uterine cavity. Endometrial mass exhibited heterogeneous high intensity on T2-weighted magnetic resonance (MR) images, and diffusion-weighted MR images revealed high intensity throughout the tumor, consistent with malignancy. LCNEC is a highly malignant neoplasm without particular findings in terms of diagnostic imaging and pathology, so its preoperative definitive diagnosis is very difficult. However, when laboratory test, pathologic diagnosis and MR imaging suggest a poorly differentiated uterine malignancy, positron emission tomography-computed tomography scan should be performed as a general assessment to help with diagnosis.

No MeSH data available.


Related in: MedlinePlus