J Obstet Gynaecol Downloaded from informahealthcare.com by Chulalongkorn University on 12/26/14 For personal use only.
Gynaecology Case Reports She was diagnosed with metastatic choriocarcinoma of brain and lungs. The differentials had been leptomeningeal disease and paraneoplastic disease. Emergency dose etoposide/cisplatin (EP) treatment was commenced, followed by alternating cycles of etoposide, methotrexate, actinomycin D, cyclophosphamide and vincristine (EMA-CO). Approximately 4 weeks later, her serum hCG was 600 mIU/l, with marked clinical improvement. She was discharged in mid-December 2011, with monthly appointments for continuing chemotherapy. By mid-January 2012, her serum hCG was 8 mIU/l. Post-treatment scans in May 2012 showed the uterus significantly reduced in size. Both lung lesions were a size accountable by residual scar tissue alone. An MRI of the brain demonstrated diffuse signal changes compatible with the effects of chemotherapy, with scant evidence of residual metastatic disease. Measurements of urine hCG continued at biweekly intervals, into 2013. In the UK, treatment priorities for high-risk gestational trophoblastic disease are early detection of CNS lesions and early treatment with multiagent chemotherapy, with intrathecal methotrexate if CNS lesions are considered likely (Athanassiou et al. 1983). A landmark study at Charing Cross confirmed the efficacy of etoposide, methotrexate, actinomycin D, cyclophosphamide and vincristine protocol (EMA-CO) (Newlands et al. 1991), and this combination remains first-line treatment. After primary evacuation of gestational trophoblastic disease, spontaneous regression is the norm, occurring in 75% in one study (Kerkmeijer et al. 2007) and over 80% within a year in another (Lurain et al. 1983). Serum hCG correlates with tumour bulk, so that serial titres track disease regression. Once serum hCG normalises, disease recurrence is rare: 1 year after a normal level, the risk of recurrence is estimated at ⬍ 1% (Soper 2006). Regrettably, our patient disengaged from follow-up and it is impossible to say whether her serum hCG normalised. But a probability of persistent disease can be quantified based on the rate of decline of serum hCG or its absolute level at a point in time (Wolfberg et al. 2005). A value of 2,578 mIU/l, 8 weeks after evacuation predicts disease persistence as a more likely outcome than complete regression. Also, levels fell slowly, so that serum hCG was relatively high 6 months after evacuation (136 mIU/l).
Conclusion Choriocarcinoma is rare and CNS manifestations uncommon. Metastatic choriocarcinoma of the brain 13 years after an index pregnancy is exceptional. Our case is a salutary lesson on the importance of vigilance following primary evacuation of a molar pregnancy. Patients might be motivated to attend follow-up when the risk of persistent disease is quantified. Patients undergoing treatment for molar pregnancy (the highest risk group for choriocarcinoma) should be counselled regarding potential complications of the disease. A urinary pregnancy test is a sensitive and inexpensive method of screening for this cancer, as well as other hCG-releasing tumours that are exquisitely chemosensitive and carry a favourable prognosis when recognised and treated early. Finally, this case highlights the risks of a patient’s preoccupation with complementary therapy in high-risk situations. Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
References Athanassiou A, Begent RH, Newlands ES et al. 1983. Central nervous system metastases of choriocarcinoma: 23 years’ experience at Charing Cross Hospital. Cancer 52:1728–1735. Cagayan MSFS, Lu-Lasala LR. 2006. Management of gestational trophoblastic neoplasia with metastasis to the central nervous system: A 12 year review at the Philippine General Hospital. Journal of Reproductive Medicine 51: 785–792. Di Cintio E, Parazzini F, Rosa C et al. 1997. The epidemiology of gestational trophoblastic disease. General and Diagnostic Pathology 143:103–108. Kerkmeijer LG, Wielsma S, Masuger LF et al. 2007. Recurrent gestational trophoblastic disease after hCG normalization following hydatidiform mole in the Netherlands. Gynecologic Oncology 106:142–146.
105
Lurain JR, Brewer JI, Torok EE et al. 1983. Natural history of hydatidiform mole after primary evacuation. American Journal of Obstetrics and Gynecology 145:591–595. Menegaz RA, Resende AD, da Silva CS et al. 2004. Metastasis of choriocarcinoma to lumbar and sacral column. European Journal of Obstetrics, Gynaecology, and Reproductive Biology 113:110–113. Neubauer NL, Latif N, Kalakota K et al. 2012. Brain metastasis in gestational trophoblastic neoplasia: An update. Journal of Reproductive Medicine 57: 288–292. Newlands ES, Bagshawe KD, Begent RH et al. 1991. Results with the EMA/CO (etoposide, methotrexate, actinomycin D, cyclophosphamide, vincristine. regimen in high risk gestational trophoblastic tumours, 1979 to 1989. British Journal of Obstetrics and Gynaecology 98:550–557. Soper JT. 2006. Gestational trophoblastic disease. Obstetrics and Gynecology 176–187. Wolfberg AJ, Berkowitz RS, Goldstein DP et al. 2005. Post-evacuation hCG levels and risk of gestational trophoblastic neoplasia in women with complete molar pregnancy. Obstetrics and Gynecology 106:548–552.
Caesarean scar ectopic pregnancy successfully treated with methotrexate and mifepristone M. T. Gómez García, E. Ruiz Sánchez, G. Aguarón Benítez, J. Nogueira García, C. Callejón Rodríguez & G. González Merlo Department of Obstetrics and Gynecology, Hospital and University Complex of Albacete (SESCAM), Department of Medical Sciences, School of Medicine, Universidad de Castilla-La Mancha, Albacete, Spain DOI: 10.3109/01443615.2014.930106 Correspondence: M. T. Gómez García, Department of Obstetrics and Gynecology, Hospital and University Complex of Albacete, C/ Hermanos Falcó, n° 37, Secretaría de Ginecología, 5ª planta, 02006 Albacete, Spain. E-mail: [email protected]
Introduction Caesarean scar ectopic pregnancy (CSEP) is a rare gynaecological entity with potentially serious consequences for the patient’s reproductive future. Its incidence is not well known, although the latest data describe ranges of 1:2,500–1:8,000 of pregnancies achieved after caesarean delivery (Uysal et al. 2013). Global figures are unquestionably climbing due to the steady increase in caesarean rates worldwide. Because CSEP is rare, there is no consensus regarding the best treatment. A literature search found only a few case reports and small case series; hence, the criteria cannot be unified. We describe a patient diagnosed with CSEP who was successfully treated with methotrexate and mifepristone. The patient gave written consent to receive the treatment and for publication of this report.
Case report A 35-year-old woman with a previous caesarean delivery due to breech birth and recurrent miscarriage, successfully underwent assisted reproduction techniques (in-vitro fertilisation-intracytoplasmic sperm injection) and came to the outpatient clinic for a routine check-up in week 5 ⫹ 3. The patient was asymptomatic, with a normal examination and weight of 53 kg. An ultrasound showed a gestational sac of 9 ⫻ 6 mm in the scar area of the previous caesarean delivery. The gestational sac contained a small yolk sac and embryo with no heartbeat (Figure 1). The ultrasound also showed an empty uterus with a clearly visualised endometrium, empty cervical canal, normal ovaries and empty pouch of Douglas. Colour Doppler flow imaging included characteristics of high velocity and low impedance. BetahCG was 1,716 mU/ml. The patient was given 50 mg of methotrexate i.m. and 600 mg of oral mifepristone. In a subsequent follow-up, the β-hCG levels were 1,477 mU/ml on day 4 and 1,097 mU/ml on day
106
Gynaecology Case Reports
Diagnostic and therapeutic dilemma in the management of primary small-cell neuroendocrine tumour of the cervix N. Singh, P. Varshney, R. Tripathi & Y. M. Mala Department of Obstetrics and Gynaecology, Maulana Azad Medical College and associated LokNayak Hospital, New Delhi, India DOI: 10.3109/01443615.2014.930424
J Obstet Gynaecol Downloaded from informahealthcare.com by Chulalongkorn University on 12/26/14 For personal use only.
Figure 1. A gestational sac of 9 ⫻ 6 mm is visualised in the scar area of a previous caesarean delivery; in its interior, a small yolk sac and embryo with no heartbeat.
Correspondence: N. Singh, Department of Obstetrics and Gynaecology, Maulana Azad Medical College and associated LokNayak Hospital, Bahadur Shah ZafarMarg, Delhi Gate, New Delhi 110002, India. E-mail: nilanchalisingh@ gmail.com
Introduction 7; then 447 mU/ml, 98 mU/ml and ⬍ 1 mU/ml in weeks 2, 3 and 4, respectively. The patient remained asymptomatic at all times and was discharged from care after 4 weeks.
Discussion As far as we are aware, this is the first case of CSEP successfully treated with methotrexate i.m. and oral mifepristone as a single dose. This treatment has already been used by our research group, as well as by others for non-tubal ectopic (cornual and cervical) pregnancies with a poor prognosis. The mechanism of action of the combination of the two drugs in the resolution of ectopic pregnancy is uncertain. It has been speculated that the antitrophoblastic effect of methotrexate is potentiated with the antidecidual action of mifepristone, which could cause the destruction of ectopic trophoblasts (Gómez García et al. 2012). In 2006, Storgaard et al. reported a CSEP treated with methotrexate and mifepristone, but the combination was not effective, and a transvaginal intrathoracic injection of potassium chloride was required for resolution. The literature describes various therapeutic strategies with good outcomes. Recently, the American Society for Reproductive Medicine (Pfeifer et al. 2013) highlighted the role of systemic and/ or local methotrexate with and without subsequent dilation and curettage. It also mentions surgical options (dilation and curettage and trophoblastic excision via either laparotomy or laparoscopy) (Pfeifer et al. 2013). Other options include a wait-and-see attitude, local administration of embryocides, hysteroscopic coagulation and embolisation of uterine arteries, either isolated or combined (Litwicka and Greco 2011). Early diagnosis is essential, and treatment should be conservative and individualised. Methotrexate plus mifepristone is a therapeutic option that could be effective. Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
References Gómez García MT, Aguarón Benitez G, Barberá Belda B et al. 2012. Medical therapy (methotrexate and mifepristone) alone or in combination with another type of therapy for the management of cervical or interstitial ectopic pregnancy. European Journal of Obstetrics, Gynecology, and Reproductive Biology 165:77–81. Litwicka K, Greco E. 2011. Caesarean scar pregnancy: a review of management options. Current Opinion in Obstetrics and Gynecology 23:415–421. Pfeifer S, Goldberg J, Lobo R et al. 2013. Practice Committee of the American Society for Reproductive Medicine. Medical treatment of ectopic pregnancy: a committee opinion. Fertility and Sterility 100:638–644. Storgaard T, Frandsen KH, Lauszus FF. 2006. Caesarean section scar pregnancy – various treatment alternatives. Ugeskrift for Laeger 168:2820–2821. Uysal F, Uysal A, Adam G. 2013. Cesarean scar pregnancy: diagnosis, management, and follow-up. Journal of Ultrasound in Medicine 32:1295–1300.
Neuroendocrine tumour of the cervix is a rare tumour accounting for 3% of all cervical malignancies (Abeler et al. 1994). It is associated with HPV 18 or 16 and develops from either neuroendocrine cells occurring in the normal endocervix or from stimulated multipotential reserve cells of the endocervical epithelium (Siriaunkgul et al. 2012). This tumour has been documented to be resistant to all forms of treatment and therefore, has a poor prognosis (Bifulco et al. 2009). Due to the paucity of literature, with only a few reported cases, no consensus exists regarding the treatment modality of choice. We report a 30-year-old woman, diagnosed with neuroendocrine tumour of the cervix, with lung metastasis.
Case report A 30-year-old, female non-smoker presented to our cancer clinic with complaints of inter-menstrual bleeding for 3 months. General and abdominal examinations were normal. Speculum visualisation showed an exophytic growth of 2 ⫻ 2 cm size on the cervix. Vaginal examination revealed friable growth felt on cervix, which bled on touch. Bilateral parametria had no tumoral involvement. Cervical biopsy from the growth was suggestive of small-cell neuroendocrine carcinoma. Cells were small-cell in type with condensed nuclear chromatin, arranged in nests and strands. Chromogranin, a prognostic marker of neuroendocrine tumour, was positive. A chest X-ray showed homogenous well-defined rounded opacity in the right paracardiac region Figure 1(a). Contrast enhanced computed tomography (CECT) of the thorax revealed a well-defined, round, hypodense lesion in right middle lobe of the lung, likely to be metastatic in nature. CECT head and abdomen showed no abnormality. CECT-scan of the pelvis revealed a bulky cervix without parametrial invasion. No lymphadenopathy was noted. The patient refused to undergo any diagnostic or therapeutic procedure for the chest lesion. Due to the rare nature of the malignancy, no treatment guidelines exist. In view of suspicion of metastatic chest lesion (Stage IV disease), the initial treatment modality considered was chemo-radiation, but, as there was no local spread clinically or radiologically, we opted for neoadjuvant chemotherapy and radical surgery. The patient received four cycles of neoadjuvant chemotherapy (etoposide, carboplatin, ifosfamide). Modified radical hysterectomy with bilateral pelvic lymphadenectomy was performed Figure 1(b). The histopathology of the specimen confirmed the diagnosis of neuroendocrine carcinoma Figure 1(c,d). Parametrium, lymph nodes and margins were free of tumour. Post-surgery chest X-ray showed the same lesion as before. A PET scan was performed, which showed low activity in the chest lesion but there were high activity areas noted in the pelvis. In view of these findings, the decision for radiation therapy was taken. The woman received 200 cGy daily, in 25 fractions, up to a total of 5,000 cGy external beam radiation therapy. The patient remained asymptomatic during 18 months follow-up. The chest X-ray performed 10 months after treatment showed no change in the size of the lesion.
Gynaecology Case Reports She was diagnosed with metastatic choriocarcinoma of brain and lungs. The differentials had been leptomeningeal disease and paraneoplastic disease. Emergency dose etoposide/cisplatin (EP) treatment was commenced, followed by alternating cycles of etoposide, methotrexate, actinomycin D, cyclophosphamide and vincristine (EMA-CO). Approximately 4 weeks later, her serum hCG was 600 mIU/l, with marked clinical improvement. She was discharged in mid-December 2011, with monthly appointments for continuing chemotherapy. By mid-January 2012, her serum hCG was 8 mIU/l. Post-treatment scans in May 2012 showed the uterus significantly reduced in size. Both lung lesions were a size accountable by residual scar tissue alone. An MRI of the brain demonstrated diffuse signal changes compatible with the effects of chemotherapy, with scant evidence of residual metastatic disease. Measurements of urine hCG continued at biweekly intervals, into 2013. In the UK, treatment priorities for high-risk gestational trophoblastic disease are early detection of CNS lesions and early treatment with multiagent chemotherapy, with intrathecal methotrexate if CNS lesions are considered likely (Athanassiou et al. 1983). A landmark study at Charing Cross confirmed the efficacy of etoposide, methotrexate, actinomycin D, cyclophosphamide and vincristine protocol (EMA-CO) (Newlands et al. 1991), and this combination remains first-line treatment. After primary evacuation of gestational trophoblastic disease, spontaneous regression is the norm, occurring in 75% in one study (Kerkmeijer et al. 2007) and over 80% within a year in another (Lurain et al. 1983). Serum hCG correlates with tumour bulk, so that serial titres track disease regression. Once serum hCG normalises, disease recurrence is rare: 1 year after a normal level, the risk of recurrence is estimated at ⬍ 1% (Soper 2006). Regrettably, our patient disengaged from follow-up and it is impossible to say whether her serum hCG normalised. But a probability of persistent disease can be quantified based on the rate of decline of serum hCG or its absolute level at a point in time (Wolfberg et al. 2005). A value of 2,578 mIU/l, 8 weeks after evacuation predicts disease persistence as a more likely outcome than complete regression. Also, levels fell slowly, so that serum hCG was relatively high 6 months after evacuation (136 mIU/l).
Conclusion Choriocarcinoma is rare and CNS manifestations uncommon. Metastatic choriocarcinoma of the brain 13 years after an index pregnancy is exceptional. Our case is a salutary lesson on the importance of vigilance following primary evacuation of a molar pregnancy. Patients might be motivated to attend follow-up when the risk of persistent disease is quantified. Patients undergoing treatment for molar pregnancy (the highest risk group for choriocarcinoma) should be counselled regarding potential complications of the disease. A urinary pregnancy test is a sensitive and inexpensive method of screening for this cancer, as well as other hCG-releasing tumours that are exquisitely chemosensitive and carry a favourable prognosis when recognised and treated early. Finally, this case highlights the risks of a patient’s preoccupation with complementary therapy in high-risk situations. Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
References Athanassiou A, Begent RH, Newlands ES et al. 1983. Central nervous system metastases of choriocarcinoma: 23 years’ experience at Charing Cross Hospital. Cancer 52:1728–1735. Cagayan MSFS, Lu-Lasala LR. 2006. Management of gestational trophoblastic neoplasia with metastasis to the central nervous system: A 12 year review at the Philippine General Hospital. Journal of Reproductive Medicine 51: 785–792. Di Cintio E, Parazzini F, Rosa C et al. 1997. The epidemiology of gestational trophoblastic disease. General and Diagnostic Pathology 143:103–108. Kerkmeijer LG, Wielsma S, Masuger LF et al. 2007. Recurrent gestational trophoblastic disease after hCG normalization following hydatidiform mole in the Netherlands. Gynecologic Oncology 106:142–146.
105
Lurain JR, Brewer JI, Torok EE et al. 1983. Natural history of hydatidiform mole after primary evacuation. American Journal of Obstetrics and Gynecology 145:591–595. Menegaz RA, Resende AD, da Silva CS et al. 2004. Metastasis of choriocarcinoma to lumbar and sacral column. European Journal of Obstetrics, Gynaecology, and Reproductive Biology 113:110–113. Neubauer NL, Latif N, Kalakota K et al. 2012. Brain metastasis in gestational trophoblastic neoplasia: An update. Journal of Reproductive Medicine 57: 288–292. Newlands ES, Bagshawe KD, Begent RH et al. 1991. Results with the EMA/CO (etoposide, methotrexate, actinomycin D, cyclophosphamide, vincristine. regimen in high risk gestational trophoblastic tumours, 1979 to 1989. British Journal of Obstetrics and Gynaecology 98:550–557. Soper JT. 2006. Gestational trophoblastic disease. Obstetrics and Gynecology 176–187. Wolfberg AJ, Berkowitz RS, Goldstein DP et al. 2005. Post-evacuation hCG levels and risk of gestational trophoblastic neoplasia in women with complete molar pregnancy. Obstetrics and Gynecology 106:548–552.
Caesarean scar ectopic pregnancy successfully treated with methotrexate and mifepristone M. T. Gómez García, E. Ruiz Sánchez, G. Aguarón Benítez, J. Nogueira García, C. Callejón Rodríguez & G. González Merlo Department of Obstetrics and Gynecology, Hospital and University Complex of Albacete (SESCAM), Department of Medical Sciences, School of Medicine, Universidad de Castilla-La Mancha, Albacete, Spain DOI: 10.3109/01443615.2014.930106 Correspondence: M. T. Gómez García, Department of Obstetrics and Gynecology, Hospital and University Complex of Albacete, C/ Hermanos Falcó, n° 37, Secretaría de Ginecología, 5ª planta, 02006 Albacete, Spain. E-mail: [email protected]
Introduction Caesarean scar ectopic pregnancy (CSEP) is a rare gynaecological entity with potentially serious consequences for the patient’s reproductive future. Its incidence is not well known, although the latest data describe ranges of 1:2,500–1:8,000 of pregnancies achieved after caesarean delivery (Uysal et al. 2013). Global figures are unquestionably climbing due to the steady increase in caesarean rates worldwide. Because CSEP is rare, there is no consensus regarding the best treatment. A literature search found only a few case reports and small case series; hence, the criteria cannot be unified. We describe a patient diagnosed with CSEP who was successfully treated with methotrexate and mifepristone. The patient gave written consent to receive the treatment and for publication of this report.
Case report A 35-year-old woman with a previous caesarean delivery due to breech birth and recurrent miscarriage, successfully underwent assisted reproduction techniques (in-vitro fertilisation-intracytoplasmic sperm injection) and came to the outpatient clinic for a routine check-up in week 5 ⫹ 3. The patient was asymptomatic, with a normal examination and weight of 53 kg. An ultrasound showed a gestational sac of 9 ⫻ 6 mm in the scar area of the previous caesarean delivery. The gestational sac contained a small yolk sac and embryo with no heartbeat (Figure 1). The ultrasound also showed an empty uterus with a clearly visualised endometrium, empty cervical canal, normal ovaries and empty pouch of Douglas. Colour Doppler flow imaging included characteristics of high velocity and low impedance. BetahCG was 1,716 mU/ml. The patient was given 50 mg of methotrexate i.m. and 600 mg of oral mifepristone. In a subsequent follow-up, the β-hCG levels were 1,477 mU/ml on day 4 and 1,097 mU/ml on day
106
Gynaecology Case Reports
Diagnostic and therapeutic dilemma in the management of primary small-cell neuroendocrine tumour of the cervix N. Singh, P. Varshney, R. Tripathi & Y. M. Mala Department of Obstetrics and Gynaecology, Maulana Azad Medical College and associated LokNayak Hospital, New Delhi, India DOI: 10.3109/01443615.2014.930424
J Obstet Gynaecol Downloaded from informahealthcare.com by Chulalongkorn University on 12/26/14 For personal use only.
Figure 1. A gestational sac of 9 ⫻ 6 mm is visualised in the scar area of a previous caesarean delivery; in its interior, a small yolk sac and embryo with no heartbeat.
Correspondence: N. Singh, Department of Obstetrics and Gynaecology, Maulana Azad Medical College and associated LokNayak Hospital, Bahadur Shah ZafarMarg, Delhi Gate, New Delhi 110002, India. E-mail: nilanchalisingh@ gmail.com
Introduction 7; then 447 mU/ml, 98 mU/ml and ⬍ 1 mU/ml in weeks 2, 3 and 4, respectively. The patient remained asymptomatic at all times and was discharged from care after 4 weeks.
Discussion As far as we are aware, this is the first case of CSEP successfully treated with methotrexate i.m. and oral mifepristone as a single dose. This treatment has already been used by our research group, as well as by others for non-tubal ectopic (cornual and cervical) pregnancies with a poor prognosis. The mechanism of action of the combination of the two drugs in the resolution of ectopic pregnancy is uncertain. It has been speculated that the antitrophoblastic effect of methotrexate is potentiated with the antidecidual action of mifepristone, which could cause the destruction of ectopic trophoblasts (Gómez García et al. 2012). In 2006, Storgaard et al. reported a CSEP treated with methotrexate and mifepristone, but the combination was not effective, and a transvaginal intrathoracic injection of potassium chloride was required for resolution. The literature describes various therapeutic strategies with good outcomes. Recently, the American Society for Reproductive Medicine (Pfeifer et al. 2013) highlighted the role of systemic and/ or local methotrexate with and without subsequent dilation and curettage. It also mentions surgical options (dilation and curettage and trophoblastic excision via either laparotomy or laparoscopy) (Pfeifer et al. 2013). Other options include a wait-and-see attitude, local administration of embryocides, hysteroscopic coagulation and embolisation of uterine arteries, either isolated or combined (Litwicka and Greco 2011). Early diagnosis is essential, and treatment should be conservative and individualised. Methotrexate plus mifepristone is a therapeutic option that could be effective. Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
References Gómez García MT, Aguarón Benitez G, Barberá Belda B et al. 2012. Medical therapy (methotrexate and mifepristone) alone or in combination with another type of therapy for the management of cervical or interstitial ectopic pregnancy. European Journal of Obstetrics, Gynecology, and Reproductive Biology 165:77–81. Litwicka K, Greco E. 2011. Caesarean scar pregnancy: a review of management options. Current Opinion in Obstetrics and Gynecology 23:415–421. Pfeifer S, Goldberg J, Lobo R et al. 2013. Practice Committee of the American Society for Reproductive Medicine. Medical treatment of ectopic pregnancy: a committee opinion. Fertility and Sterility 100:638–644. Storgaard T, Frandsen KH, Lauszus FF. 2006. Caesarean section scar pregnancy – various treatment alternatives. Ugeskrift for Laeger 168:2820–2821. Uysal F, Uysal A, Adam G. 2013. Cesarean scar pregnancy: diagnosis, management, and follow-up. Journal of Ultrasound in Medicine 32:1295–1300.
Neuroendocrine tumour of the cervix is a rare tumour accounting for 3% of all cervical malignancies (Abeler et al. 1994). It is associated with HPV 18 or 16 and develops from either neuroendocrine cells occurring in the normal endocervix or from stimulated multipotential reserve cells of the endocervical epithelium (Siriaunkgul et al. 2012). This tumour has been documented to be resistant to all forms of treatment and therefore, has a poor prognosis (Bifulco et al. 2009). Due to the paucity of literature, with only a few reported cases, no consensus exists regarding the treatment modality of choice. We report a 30-year-old woman, diagnosed with neuroendocrine tumour of the cervix, with lung metastasis.
Case report A 30-year-old, female non-smoker presented to our cancer clinic with complaints of inter-menstrual bleeding for 3 months. General and abdominal examinations were normal. Speculum visualisation showed an exophytic growth of 2 ⫻ 2 cm size on the cervix. Vaginal examination revealed friable growth felt on cervix, which bled on touch. Bilateral parametria had no tumoral involvement. Cervical biopsy from the growth was suggestive of small-cell neuroendocrine carcinoma. Cells were small-cell in type with condensed nuclear chromatin, arranged in nests and strands. Chromogranin, a prognostic marker of neuroendocrine tumour, was positive. A chest X-ray showed homogenous well-defined rounded opacity in the right paracardiac region Figure 1(a). Contrast enhanced computed tomography (CECT) of the thorax revealed a well-defined, round, hypodense lesion in right middle lobe of the lung, likely to be metastatic in nature. CECT head and abdomen showed no abnormality. CECT-scan of the pelvis revealed a bulky cervix without parametrial invasion. No lymphadenopathy was noted. The patient refused to undergo any diagnostic or therapeutic procedure for the chest lesion. Due to the rare nature of the malignancy, no treatment guidelines exist. In view of suspicion of metastatic chest lesion (Stage IV disease), the initial treatment modality considered was chemo-radiation, but, as there was no local spread clinically or radiologically, we opted for neoadjuvant chemotherapy and radical surgery. The patient received four cycles of neoadjuvant chemotherapy (etoposide, carboplatin, ifosfamide). Modified radical hysterectomy with bilateral pelvic lymphadenectomy was performed Figure 1(b). The histopathology of the specimen confirmed the diagnosis of neuroendocrine carcinoma Figure 1(c,d). Parametrium, lymph nodes and margins were free of tumour. Post-surgery chest X-ray showed the same lesion as before. A PET scan was performed, which showed low activity in the chest lesion but there were high activity areas noted in the pelvis. In view of these findings, the decision for radiation therapy was taken. The woman received 200 cGy daily, in 25 fractions, up to a total of 5,000 cGy external beam radiation therapy. The patient remained asymptomatic during 18 months follow-up. The chest X-ray performed 10 months after treatment showed no change in the size of the lesion.
