Journal of Obstetrics and Gynaecology, April 2015; 35: 314–328 © 2015 Informa UK, Ltd. ISSN 0144-3615 print/ISSN 1364-6893 online
GYNAECOLOGY CASE REPORTS
Small cell neuroendocrine carcinoma of cervix and leiomyoma between the vagina and rectum
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S. Sheth1, D. Shende2 & S. Arneja3 1Breach Candy Hospital, Saifee Hospital, Sir Hurkisondas Hospital and Sheth Maternity and Gynaecological Nursing Home, 2Sheth Maternity and Gynecological Nursing Home and 3Department of Surgical and Cytology, Breach Candy Hospital, Mumbai, India
DOI: 10.3109/01443615.2014.940295 Correspondence: D. Shende, 2/2, Navjivan Society, Lamington Road, Mumbai 400008, India. E-mail: [email protected]
Case report A 45-year-old woman, P2L2, presented with an increase in the periodicity of menses and intermenstrual bleeding. Speculum examination, revealed an unhealthy cervix but without frank growth. On vaginal examination, a lemon sized, firm non-tender fibroma-like
mass was felt between the vagina and rectum. It occupied the space below the cervix and behind the middle third of the vagina. Rectal examination revealed that the rectum was free from the mass, which ruled out rectal pathology. Biopsy of the unhealthy cervix was performed, as she had already undergone biopsy of the fibroma-like mass, suggesting low-grade fibrosarcoma, before being seen by us. The biopsy of the cervix suggested small cell neuroendocrine carcinoma of the cervix. The surgeries of the two ailments were combined: the mass was excised vaginally by commencing the incision from the mucocutaneous junction upwards until the entire mass was well felt, enucleated and sent for frozen histopathology study. The rectal finger aided enucleation. After histopathological confirmation that the mass was benign, it was followed by Wertheim’s radical hysterectomy. The liver was palpated and inspected by a laparoscope from the Pfannenstiel’s operative wound and found to be normal. Para-aortic lymph nodes were not enlarged. Postoperative follow-up was normal. Final histopathology reports for the mass were suggestive of benign leiomyoma (Figure 1a, b) and that of the cervix revealed small cell neuroendocrine carcinoma of the cervix (SCNCC) (Figure 1c, d). Myometrium, parametrium, vagina, tubes, ovaries and lymph nodes were free of metastasis. The lesional cells showed strong immunopositivity for CD56 and synaptophysin and focal positivity for chromogranin. CK5/6 was negative; P63 positivity was noted in small percentage of cells; Mib-1 labelling index was approximately 80%. The adjacent glands
Figure 1. (a, b) Mass between the vagina and the rectum. Fascicles of benign smooth muscle bundles. Cells have elongated regular blunt ended nuclei. Mitotic activity/ atypia is not discernable. (H&E; a, ⫻ 20; b, ⫻ 40). (c) Small cell carcinoma of the cervix. (H&E ⫻ 20). (d) Adenocarcinoma in situ was noted. Two of the glands on the left show adenocarcinoma in situ. The morphology of abnormal glands is compared with the large normal gland on the right. (H&E ⫻ 20).
J Obstet Gynaecol Downloaded from informahealthcare.com by Nyu Medical Center on 05/19/15 For personal use only.
Gynaecology Case Reports 315 showed adenocarcinoma in situ (Figure 1d). The final diagnosis was SCNCC; FIGO stage IB1. The patient was then referred for further treatment to the medical oncologist.
Correspondence: A. Karabulut, Onelya Evleri, A-4 Yenişehir, 20125 Denizli, Turkey. E-mail: [email protected]
Discussion
Granulocytic sarcoma (GS), which is also known as chloroma, is a solid collection of leukaemic cells occurring outside of the bone marrow in acute myelogenous leukaemia (AML) (Rappaport 1966). This disease can be observed in patients with acute leukaemia or chronic myeloid leukaemia in the blastic phase (Rappaport 1966). Granulocytic sarcoma is seen in 3–5% of patients with acute myelogenous leukaemia. It is a disease which is encountered in 0.7/million in the juvenile population and 2/million in the adult population (Neiman et al. 1981). In this report, we present a case of GS of the uterine cervix with heavy vaginal bleeding, resulting in unnecessary surgery. We then discuss the effect of surgery on the disease progress in light of the current literature.
Neuroendocrine carcinoma of the uterine cervix (NCC) is rare, highly aggressive and prone to metastasis. SCNCC is most common among the NCC and comprises 1–3% of cervical carcinoma whose aetiology and predisposing risk factors are poorly understood (Van Nagell et al. 1988). SCNCC exhibits aggressive behaviour and poor prognosis, even after multimodal therapy, as very limited data is available for understanding of both management and effective therapy. The occurrence of leiomyoma between the vagina and rectum is rare. Its aetiology is unknown, although there is much speculation. A MEDLINE search for articles with keywords ‘small cell neuroendocrine carcinoma of cervix’ in the last 10 years yielded eight reports, and a search of the entire English literature for leiomyoma between vagina and rectum yielded none. Some studies have demonstrated a relationship between HPV infection and NCC of the cervix (Stoler et al. 1991). In 2011, the Society of Gynaecological Oncology issued a clinical document summarising the available literature on NCC of the female reproductive tract. In a recent study on the 5-year survival rate of patients with FIGO stage IB1, the survival rate was 50–60%, which was significantly poorer than the 90% rate for patients with stage IB1 of squamous cell carcinoma (Viswanathan et al. 2004). Recent retrospective data suggests that surgery may improve the clinical outcome and radiation is used with concurrent etoposide/ cisplatinum. For advanced stage disease, metastatic sites are treated with platinum-based combination chemotherapy (Dowell 2010). SCNCC have a reported 5-year survival of 36% (Sevin et al. 1996). Periodic full body imaging with CT or PET/CT to evaluate neurological symptoms, mental status changes or pulmonary metastases are necessary. The histopathology report is vital for the appropriate management and offers relief to the suffering woman. For the gynaecologist and oncologist, a lapse in diagnosis or treatment can prove disastrous. This case is reported for its rarity in the realms of cervical malignancy, as well as the unusual site for occurrence of a leiomyoma, between the vagina and rectum, and the concurrent presence of both.
Introduction
Case report A 59-year-old woman presented to our gynaecology department with postmenopausal bleeding. A pelvic examination showed an 8–10 cm mass within an enlarged cervix. Her past medical history revealed acute myeloblastic leukaemia (AML-M4) 14 years ago, which relapsed 10 years ago. She was successfully treated with chemotherapy at both times, and she had now been in remission for 10 years. Laboratory tests were within normal limits. Slightly increased spleen size and a 12 ⫻ 13 ⫻ 10 cm cervical mass were detected on sonography. Cervical biopsy was obtained from the cervical mass. However, we had to
Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
References Dowell JE. 2010. Small cell lung cancer: are we making progress? American Journal of the Medical Sciences 339:68–76. Sevin BU, Method MW, Nadji M et al. 1996. Efficacy of radical hysterectomy as treatment for patients with small cell carcinoma of the cervix. Cancer 77:1489–1493. Stoler MH, Mills SE, Gersell DJ et al. 1991. Small-cell neuroendocrine carcinoma of the cervix. A human papillomavirus type 18-associated cancer. American Journal of Surgical Pathology 15:28–32. Van Nagell JR, Powell DE, Gallion HH et al. 1988. Small cell carcinoma of the uterine cervix. Cancer 62:1586–1593. Viswanathan AN, Deavers MT, Jhingran A et al. 2004. Small cell neuroendocrine carcinoma of the cervix: outcome and patterns of Recurrence. Gynecologic Oncology 93:27–33.
Granulocytic sarcoma of the cervix: Is hysterectomy necessary? I. Gunyeli1, S. A. Kose1, O. Ozkaya1, A. G. Kose1, A. Karabulut3 & N. Kapucuoglu2 1Department of Obstetrics and Gynecology, 2Department of Pathology, Suleyman Demirel University, Faculty of Medicine, Isparta and 3Department of Obstetrics and Gynecology, Pamukkale University, Faculty of Medicine, Denizli, Turkey
DOI: 10.3109/01443615.2014.940296
Figure 1. (a) Postoperative appearance of the uterus, ovaries and enlarged cervix with tumoral cells. (b) Diffuse infiltration of small atypical lymphoid cells and sporadically localised large nucleated blastic cells (some of them are multinucleated and large eosinophilic cytoplasm) are seen in the histopathological sections (H&E ⫻ 200).
GYNAECOLOGY CASE REPORTS
Small cell neuroendocrine carcinoma of cervix and leiomyoma between the vagina and rectum
J Obstet Gynaecol Downloaded from informahealthcare.com by Nyu Medical Center on 05/19/15 For personal use only.
S. Sheth1, D. Shende2 & S. Arneja3 1Breach Candy Hospital, Saifee Hospital, Sir Hurkisondas Hospital and Sheth Maternity and Gynaecological Nursing Home, 2Sheth Maternity and Gynecological Nursing Home and 3Department of Surgical and Cytology, Breach Candy Hospital, Mumbai, India
DOI: 10.3109/01443615.2014.940295 Correspondence: D. Shende, 2/2, Navjivan Society, Lamington Road, Mumbai 400008, India. E-mail: [email protected]
Case report A 45-year-old woman, P2L2, presented with an increase in the periodicity of menses and intermenstrual bleeding. Speculum examination, revealed an unhealthy cervix but without frank growth. On vaginal examination, a lemon sized, firm non-tender fibroma-like
mass was felt between the vagina and rectum. It occupied the space below the cervix and behind the middle third of the vagina. Rectal examination revealed that the rectum was free from the mass, which ruled out rectal pathology. Biopsy of the unhealthy cervix was performed, as she had already undergone biopsy of the fibroma-like mass, suggesting low-grade fibrosarcoma, before being seen by us. The biopsy of the cervix suggested small cell neuroendocrine carcinoma of the cervix. The surgeries of the two ailments were combined: the mass was excised vaginally by commencing the incision from the mucocutaneous junction upwards until the entire mass was well felt, enucleated and sent for frozen histopathology study. The rectal finger aided enucleation. After histopathological confirmation that the mass was benign, it was followed by Wertheim’s radical hysterectomy. The liver was palpated and inspected by a laparoscope from the Pfannenstiel’s operative wound and found to be normal. Para-aortic lymph nodes were not enlarged. Postoperative follow-up was normal. Final histopathology reports for the mass were suggestive of benign leiomyoma (Figure 1a, b) and that of the cervix revealed small cell neuroendocrine carcinoma of the cervix (SCNCC) (Figure 1c, d). Myometrium, parametrium, vagina, tubes, ovaries and lymph nodes were free of metastasis. The lesional cells showed strong immunopositivity for CD56 and synaptophysin and focal positivity for chromogranin. CK5/6 was negative; P63 positivity was noted in small percentage of cells; Mib-1 labelling index was approximately 80%. The adjacent glands
Figure 1. (a, b) Mass between the vagina and the rectum. Fascicles of benign smooth muscle bundles. Cells have elongated regular blunt ended nuclei. Mitotic activity/ atypia is not discernable. (H&E; a, ⫻ 20; b, ⫻ 40). (c) Small cell carcinoma of the cervix. (H&E ⫻ 20). (d) Adenocarcinoma in situ was noted. Two of the glands on the left show adenocarcinoma in situ. The morphology of abnormal glands is compared with the large normal gland on the right. (H&E ⫻ 20).
J Obstet Gynaecol Downloaded from informahealthcare.com by Nyu Medical Center on 05/19/15 For personal use only.
Gynaecology Case Reports 315 showed adenocarcinoma in situ (Figure 1d). The final diagnosis was SCNCC; FIGO stage IB1. The patient was then referred for further treatment to the medical oncologist.
Correspondence: A. Karabulut, Onelya Evleri, A-4 Yenişehir, 20125 Denizli, Turkey. E-mail: [email protected]
Discussion
Granulocytic sarcoma (GS), which is also known as chloroma, is a solid collection of leukaemic cells occurring outside of the bone marrow in acute myelogenous leukaemia (AML) (Rappaport 1966). This disease can be observed in patients with acute leukaemia or chronic myeloid leukaemia in the blastic phase (Rappaport 1966). Granulocytic sarcoma is seen in 3–5% of patients with acute myelogenous leukaemia. It is a disease which is encountered in 0.7/million in the juvenile population and 2/million in the adult population (Neiman et al. 1981). In this report, we present a case of GS of the uterine cervix with heavy vaginal bleeding, resulting in unnecessary surgery. We then discuss the effect of surgery on the disease progress in light of the current literature.
Neuroendocrine carcinoma of the uterine cervix (NCC) is rare, highly aggressive and prone to metastasis. SCNCC is most common among the NCC and comprises 1–3% of cervical carcinoma whose aetiology and predisposing risk factors are poorly understood (Van Nagell et al. 1988). SCNCC exhibits aggressive behaviour and poor prognosis, even after multimodal therapy, as very limited data is available for understanding of both management and effective therapy. The occurrence of leiomyoma between the vagina and rectum is rare. Its aetiology is unknown, although there is much speculation. A MEDLINE search for articles with keywords ‘small cell neuroendocrine carcinoma of cervix’ in the last 10 years yielded eight reports, and a search of the entire English literature for leiomyoma between vagina and rectum yielded none. Some studies have demonstrated a relationship between HPV infection and NCC of the cervix (Stoler et al. 1991). In 2011, the Society of Gynaecological Oncology issued a clinical document summarising the available literature on NCC of the female reproductive tract. In a recent study on the 5-year survival rate of patients with FIGO stage IB1, the survival rate was 50–60%, which was significantly poorer than the 90% rate for patients with stage IB1 of squamous cell carcinoma (Viswanathan et al. 2004). Recent retrospective data suggests that surgery may improve the clinical outcome and radiation is used with concurrent etoposide/ cisplatinum. For advanced stage disease, metastatic sites are treated with platinum-based combination chemotherapy (Dowell 2010). SCNCC have a reported 5-year survival of 36% (Sevin et al. 1996). Periodic full body imaging with CT or PET/CT to evaluate neurological symptoms, mental status changes or pulmonary metastases are necessary. The histopathology report is vital for the appropriate management and offers relief to the suffering woman. For the gynaecologist and oncologist, a lapse in diagnosis or treatment can prove disastrous. This case is reported for its rarity in the realms of cervical malignancy, as well as the unusual site for occurrence of a leiomyoma, between the vagina and rectum, and the concurrent presence of both.
Introduction
Case report A 59-year-old woman presented to our gynaecology department with postmenopausal bleeding. A pelvic examination showed an 8–10 cm mass within an enlarged cervix. Her past medical history revealed acute myeloblastic leukaemia (AML-M4) 14 years ago, which relapsed 10 years ago. She was successfully treated with chemotherapy at both times, and she had now been in remission for 10 years. Laboratory tests were within normal limits. Slightly increased spleen size and a 12 ⫻ 13 ⫻ 10 cm cervical mass were detected on sonography. Cervical biopsy was obtained from the cervical mass. However, we had to
Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
References Dowell JE. 2010. Small cell lung cancer: are we making progress? American Journal of the Medical Sciences 339:68–76. Sevin BU, Method MW, Nadji M et al. 1996. Efficacy of radical hysterectomy as treatment for patients with small cell carcinoma of the cervix. Cancer 77:1489–1493. Stoler MH, Mills SE, Gersell DJ et al. 1991. Small-cell neuroendocrine carcinoma of the cervix. A human papillomavirus type 18-associated cancer. American Journal of Surgical Pathology 15:28–32. Van Nagell JR, Powell DE, Gallion HH et al. 1988. Small cell carcinoma of the uterine cervix. Cancer 62:1586–1593. Viswanathan AN, Deavers MT, Jhingran A et al. 2004. Small cell neuroendocrine carcinoma of the cervix: outcome and patterns of Recurrence. Gynecologic Oncology 93:27–33.
Granulocytic sarcoma of the cervix: Is hysterectomy necessary? I. Gunyeli1, S. A. Kose1, O. Ozkaya1, A. G. Kose1, A. Karabulut3 & N. Kapucuoglu2 1Department of Obstetrics and Gynecology, 2Department of Pathology, Suleyman Demirel University, Faculty of Medicine, Isparta and 3Department of Obstetrics and Gynecology, Pamukkale University, Faculty of Medicine, Denizli, Turkey
DOI: 10.3109/01443615.2014.940296
Figure 1. (a) Postoperative appearance of the uterus, ovaries and enlarged cervix with tumoral cells. (b) Diffuse infiltration of small atypical lymphoid cells and sporadically localised large nucleated blastic cells (some of them are multinucleated and large eosinophilic cytoplasm) are seen in the histopathological sections (H&E ⫻ 200).
