|Year : 2022 | Volume
| Issue : 1 | Page : 28-31
Cervical sarcomatoid carcinoma – A rare case report
Monik Patel, Isha Shah, Aastha Shah, U Suryanarayana
Department of Radiation Oncology, GCRI, Ahmedabad, Gujarat, India
|Date of Submission||22-Jun-2021|
|Date of Acceptance||29-Sep-2021|
|Date of Web Publication||28-Oct-2021|
Dr. Monik Patel
Department of Radiation Oncology, GCRI, Ahmedabad, Gujarat
Source of Support: None, Conflict of Interest: None
Sarcomatoid carcinoma is a very rare morphologic entity of the female genital tract and even more rare in the cervix. Sarcomatoid carcinoma is a rare form of cancer in which the cells exhibit properties characteristic of both epithelial and mesenchymal tumors and looks like a mixture of carcinoma and sarcoma. It differs from squamous cell carcinomas of the cervix in terms of having a poorer prognosis and a short disease-free survival. The tumor is usually diagnosed at an advanced stage of presentation and is characterized by early recurrence following treatment. Surgery or radiotherapy remains the mainstay of treatment. A rare case of sarcomatoid carcinoma of the cervix in a 42-year-old female patient is reported here. The patient was examined, and biopsy from the polypoidal mass of the cervix was performed. Immunohistochemistry examination was done which favored the diagnosis as sarcomatoid carcinoma of the cervix with positivity for vimentin and P63 markers and diffuse staining of actin and negativity for CK7, CEA, ER, desmin, HMB45, MYOD1, EMA, CD10, and CK5/6. The patient was clinically defined as having International Federation of Gynecology and Obstetrics stage IIIB disease. The patient was treated by radiotherapy with a palliative approach according to clinical disease. The pathologic diagnosis of sarcomatoid carcinoma is based on histologic and immunohistochemical findings. As this entity of cervical cancer is very rare, it is difficult to draw a firm decision on disease behavior, diagnostic methods, best treatment option, and outcome. Reporting such cases might help oncologists to understand this entity of cervical cancer.
Keywords: Cervix, rare, sarcomatoid carcinoma
|How to cite this article:|
Patel M, Shah I, Shah A, Suryanarayana U. Cervical sarcomatoid carcinoma – A rare case report. J Radiat Cancer Res 2022;13:28-31
| Introduction|| |
Sarcomatoid carcinoma is a rare form of cancer in which the cells exhibit properties characteristic of both epithelial and mesenchymal tumors and looks like a mixture of carcinoma (cancer that begins in the skin or in tissues that line or cover internal organs in the body) and sarcoma (cancer of the bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissues). It can occur in multiple organs, including skin, bone, thyroid, breast, liver, pancreas, urinary tract, lung, and cervix.
Sarcomatoid carcinoma is a very rare morphologic entity of the female genital tract and even more rare in the cervix. The prognosis for women with cervical sarcomatoid carcinomas tends to be worse than that of squamous histology. Patients usually present with more advanced stages and follow a very aggressive course of disease. Because of the rarity of the disease, no clarity regarding standard diagnostic and treatment approach is available. Surgery or radiotherapy remains the mainstay of treatment. Although a good response to initial therapy was observed in the majority of cases, a subsequent recurrence is inevitable.
| Case Report|| |
A 42-year-old premenopausal woman was referred to our institute, with a 2-month history of abnormal vaginal bleeding on and off which gradually increased. It is also associated with foul-smelling whitish discharge per vagina. She also had complaints of moderate-to-severe colicky pain in the lower abdomen. General physical examination revealed severe pallor. She had a Karnofsky performance status of about 70. On per vaginal examination, ulceroproliferative growth involving the whole cervix was circumferentially noted. The growth was protruding into the vagina up to around 3 cm from the introitus. The posterior fornix was obliterated and involved. Rest all fornices were shallow. The posterior vaginal wall was involved in the upper 1 cm. Rest all vaginal walls were free. On per rectal examination, around 5 cm × 6 cm hard, fixed central bulge was palpated. The left-side parametrium was involved up to the lateral pelvic wall and the right-side parametrium was involved just one finger short of the lateral pelvic wall. Outside histopathological report of biopsy from the polypoidal mass of the cervix showed high-grade sarcoma/carcinoma. Cervix biopsy was performed in our hospital which showed possibilities of sarcoma, query being poorly differentiated carcinoma as depicted in [Figure 1]. Further immunohistochemistry examination was done which favored the diagnosis as sarcomatoid carcinoma of the cervix with positivity for vimentin and P63 markers and diffuse staining of actin and negativity for CK7, CEA, ER, desmin, HMB45, MYOD1, EMA, CD10, and CK5/6 as depicted in [Figure 2].
An ultrasonography of the abdomen and pelvis showed a 52 mm × 77 mm × 50 mm lesion involving the cervix, lower body of the uterus, upper vagina, and bilateral parametrium and indents the base of the urinary bladder with loss of fat plane. As shown in [Figure 3] and [Figure 4], computed tomography scan of the abdomen and pelvis showed a 61 mm × 86 mm × 71 mm lesion involving the cervix, lower body of the uterus, and upper vagina. The lesion abuts the posterior wall of the urinary bladder and anterior wall of the rectum with loss of fat plane. Few simple cysts were noted involving both ovaries, largest being 33 mm × 29 mm on the right side. Metastatic workup to rule out the lung, liver, and brain metastasis was done. The patient was clinically defined as having International Federation of Gynecology and Obstetrics stage IIIB disease. According to the patient's general condition and such a big lesion, the patient was planned to be treated by radiotherapy with a palliative approach. Palliative radiation therapy was planned with a total dose of 30 Gy in 10 fractions with a biologically effective dose of 39 Gy as per our institute protocol, 3 Gy per fraction per day, 5 fractions per week, with parallel-opposed anteroposterior and posteroanterior field over the pelvic region in supine position with 6-MV photon linear accelerator. At the end of the treatment, the patient had symptomatic relief as in decreased bleeding and pain. On 3-month follow-up, ultrasound abdomen and pelvis showed a decrease in size of lesion to 27 mm × 35 mm × 31 mm. The patient was not willing to take further treatment, so the best supportive care was offered thereafter.
|Figure 3: Axial image of contrast-enhanced computed tomography abdomen and pelvis|
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|Figure 4: Sagittal image of contrast-enhanced computed tomography abdomen and pelvis|
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| Discussion|| |
Squamous cell carcinoma accounts for over 85% of cervical cancer pathology; other histological types are adenocarcinoma, carcinosarcoma, lymphoma, and sarcoma. Sarcomatoid carcinoma is a rare pathological entity of the female genital tract and rarest because of the involvement of the cervix. It is seen more frequently in the aerodigestive tract and skin. In an English literature search for cervical sarcomatoid carcinomas, only 16 cases were found.,
The pathologic diagnosis of sarcomatoid carcinoma is based on histologic and immunohistochemical findings. Histologically, the squamous carcinoma presents either in a complete sarcomatoid form of spindle cells or in association with a typical squamous cell component. The immunohistochemical examination of sarcomatoid carcinoma is indicated to distinguish the epithelial differentiation from sarcoma. The histopathological diagnosis of sarcomatoid carcinoma rests upon a squamous cell carcinoma merging with a spindle cell component. Giant cells may also be seen. Cytokeratin is positive in both the spindle and squamous cell components.
Although the underlying causes associated with the development and the biologic behavior of sarcomatoid carcinomas are not fully understood, two theories have been described for the underlying basis for the development of sarcomatoid carcinomas: a monoclonal origin from a stem cell capable of divergent differentiation or a multiclonal derivation from two or more independent cell types. Several molecular studies of sarcomatoid carcinomas in different organs have shown evidence for the sarcomatoid transformation from the epithelial component, which supports a monoclonal hypothesis.
Brown et al. reported the largest series of nine cases of sarcomatoid carcinomas of the cervix, with a median disease-free interval of 4.9 months. Only one patient survived for 40 months. In this series, although all patients had a complete response to initial therapy, more than half of the patients had recurrences of the disease in less than 5 months after initial treatments. None of them responded to second-line therapy.
In literature review, it was showing a lack of p53 overexpression, and an overexpression of the phosphorylated retinoblastoma protein leads to a decrease in apoptosis when compared with the usual squamous cell cervical cancer cases., These data suggest the aggressiveness of the clinical behavior of the disease compared with the usual squamous cell histology. As this entity of cervical cancer is very rare, it is difficult to draw a firm decision on disease behavior, diagnostic methods, best treatment option, and outcome.
| Conclusion|| |
Sarcomatoid carcinomas have a more aggressive clinical behavior with a very short period of time for disease progression in comparison to squamous cell carcinomas of the cervix. Reporting such cases might help oncologists to understand this entity of cervical cancer. We do believe that sarcomatoid carcinoma should be considered a high-grade, aggressive carcinoma characterized by the development of early progression after initial therapy and failure to respond to second-line therapy, which is why a more meticulous approach at the initial presentation in the form of multimodality treatments should be considered.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]