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Case History 
A post-menopausal female in her mid-fifty's presented with lower abdominal pain for three months associated with white blood-tinged discharge per-vaginum. On per-abdomen examination a midline mass of firm consistency with smooth surface, regular border, and restricted mobility was palpable. Per-speculum examination revealed healthy cervix and vagina with cervix deviated towards left and bulging anterior fornix. Per-vaginal examination revealed a large hard mass measuring approximately 10 × 7 cm on the anterior fornix with restricted mobility with a clinical suspicion of central cervical fibroid.
USG revealed a bulky uterus completely replaced by multiple large intramural and subserosal fibroids of varying sizes forming a large myomatous mass with lobulated margins, measuring 13.6 × 8 cm.
CT scan of abdomen and pelvis showed a bulky uterus with large heterogenously enhancing mass with areas of necrosis involving the body of the uterus and extending into the left parametrium. Cervix appeared unremarkable. A presumptive differential diagnosis of carcinoma endometrium or a large uterine leiomyoma with degenerative changes causing mild left hydronephrosis was opined.
Serology for tumor markers CA19.9, AFP, and CEA were within normal limits, while CA125 was mildly elevated (38.1 U/ml).
Ecto- and endo-cervical scrape smears were received for cytological screening, which was stained with the conventional Papanicolaou stain. On microscopic examination, predominantly benign superficial and intermediate squamous epithelial cells were seen along with a few dissociated and dispersed ovoid to spindled pleomorphic cells with enlarged hyperchromatic nuclei showing anisonucleosis, coarse nuclear chromatin, and irregular nuclear membrane. Based on these features, the interpretation of atypical glandular cells- favor neoplastic (Bethesda 2014) was rendered after correlation with available sonography details, which revealed no lesion in cervico-vaginal region, and a biopsy was requested for confirmation [Figure 1] and [Figure 2].
Figure 1: Cervical scrape smears showed predominantly non-descript squamous epithelial cells along with few dissociated and dispersed ovoid to spindled pleomorphic cells (arrows) displaying enlarged hyperchromatic nuclei with anisonucleosis, coarse nuclear chromatin and irregular nuclear membrane. (a, b Papanicolaou stain x400)
Figure 2: (a-d) The cytomorphological appearance of the exfoliated cells varied from oval-spindle to elongated to plasmacytoid with anisonucleosis, coarse nuclear chromatin, irregular nuclear membrane and moderate amount of cytoplasm (a-d, Papanicolaou stain x400)Endocervical curettage specimen was then received for histopathological examination. Microscopic examination of H & E stained section showed predominant necrosis and a tiny fragment of atypical clear cells with nuclear pleomorphism and hyperchromasia arranged in sheets; inconclusive for opinion.
Next, a specimen labeled as “endometrial biopsy” was received, which on microscopic examination revealed predominantly necrosis. The viable tissue showed a tumor arranged in vague fascicles, comprised of round to oval, spindle-shaped cells with moderate to marked nuclear pleomorphism, coarse chromatin, conspicuous nucleoli, and a moderate amount of cytoplasm with frequent mitosis [Figure 3]. On immunohistochemistry, the tumor cells were positive for SMA, CD 10, desmin, and negative for PR, CD 117. Based on these features, a diagnosis of leiomyosarcoma was rendered.
Figure 3: a- Histopathological examination revealed tumour arranged in vague fascicles with areas of necrosis (Hematoxylin and eosin x100). b- The tumor cells were ovoid to spindle shaped with moderate to marked nuclear pleomorphism, coarse chromatin, and moderate amount of cytoplasm. Mitosis was frequent. (Hematoxylin and eosin x400). c-e The tumor cells were positive for desmin (IHC x400, c) and SMA (IHC x400, d) with high Ki67 (IHC x400, e) Discussion Sarcomas of the uterus account for approximately 1% of the tumors of the female genital tract.[1] On literature review, very few reports were found that described the detection and appearance of sarcoma cells on cervico-vaginal smear cytology. Massoni et al.[2] first studied the exfoliated tumor cells in leiomyosarcoma and found that very few exfoliated malignant cells were found in cervico-vaginal smears. About 6% of uterine sarcoma cases showed malignant cells in cervico-vaginal smears in their study. They also described the appearance of these exfoliated cells, which were elongated with bipolar cytoplasmic processes, cigar shaped hyperchromatic nuclei with coarse nuclear chromatin. In our case, the exfoliated cells were oval to spindle-shaped with moderate to marked nuclear pleomorphism, vesicular chromatin, conspicuous nucleoli, and a moderate amount of cytoplasm.
Other differential diagnoses that need to be considered based on the cytologic appearance of exfoliated cells include exfoliated cells from endometrial stromal sarcoma, malignant mixed Mullerian tumors, and rhabdomyosarcoma.
Exfoliation of tumor cells is predominantly seen in mass with ulceration or necrosis. The sensitivity of liquid-based cytology (LBC) in detecting endometrial carcinoma ranges from 31.9% to 89.6%.[3]
Guidos et al.[4] found a significant increase of 60% in detection rate by LBC as compared to conventional smear cytology for endometrial malignancy.
The frequency of detection of malignant cells in cervico-vaginal smears varies from 6% to 50% of sarcoma cases as described by Massoni et al.[2] and Ito et al.,[5] respectively. Only 15% of cases of leiomyosarcoma showed tumor cells in vaginal smears in a study by Wang et al.[6] and these cells had the morphological appearance of elongated/swollen end with abundant poorly defined cytoplasm, and large hyperchromatic nuclei with prominent nucleoli. In the present case, very few atypical spindle cells were noted in a dispersed manner which could have been easily overlooked if not examined carefully. Hence, diligent microscopic evaluation of cervico-vaginal smears is necessary to identify the sarcoma cells which can be present in a very small number in such smears, to avoid misdiagnosis.
Uterine leiomyosarcomas grow aggressively and have high recurrence rates even with localized disease at the time of diagnosis. For advanced disease, surgical cytoreduction with adjuvant chemotherapy is the treatment of choice.[7]
Exfoliated cells from uterine sarcomas can be detected in cervico-vaginal smears, however, the frequency is relatively low which can go undetected because of limited sampling and cytomorphologic overlap with other entities creating interpretation pitfalls even after careful examination.
Although a definite diagnosis of uterine sarcoma cannot be made on cervico-vaginal smears, a diagnosis of uterine sarcoma should be kept in mind whenever cells with unusual cytologic features are detected on smears.
Thus, familiarity with these entities is useful when unusual tumor morphology is encountered. Recognition of these rare tumors in routine cervical screening may help decrease the potential for misinterpretation and allow for more appropriate patient management.
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References 
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CheckDOI: 10.4103/joc.joc_165_21
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