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Epithelioid leiomyosarcoma of the uterine cervix: A case report and review of the literature
Gynecologic Oncology 97 (2005) 957 – 960 www.elsevier.com/locate/ygyno
Case Report
Epithelioid leiomyosarcoma of the uterine cervix: A case report and review of the literature Masafumi ToyoshimaT, Chikako Okamura, Hitoshi Niikura, Kiyoshi Ito, Nobuo Yaegashi Department of Obstetrics and Gynecology, Tohoku University Graduate School of Medicine, 1-1, Seiryo-machi, Aoba, Sendai 980-8574, Japan Received 22 November 2004 Available online 10 May 2005
Abstract Background. Epithelioid leiomyosarcomas arising from the uterine cervix are extremely rare neoplasms, with only three cases reported in the English literature. Case. A 53-year-old Japanese woman was admitted to our university hospital due to massive vaginal bleeding from a cervical tumor. A total hysterectomy with bilateral salpingo-oophorectomy was performed. Histological findings, including immunohistochemical study using desmin, SMA, cytokeratin, S-100, HMB-45, vimentin, melan-A, and CD68, led to a diagnosis of epithelioid leiomyosarcoma of the uterine cervix. The patient underwent adjuvant chemotherapy and has been disease-free for over 20 months. Conclusion. Immunohistochemical studies may be needed to differentiate among the alternative diagnoses of malignant melanoma, metastatic carcinoma, and epithelioid sarcoma. Additional cases are needed to develop optimal management strategies and to predict prognosis. D 2005 Elsevier Inc. All rights reserved. Keywords: Epithelioid leiomyosarcoma; Cervical leiomyosarcoma; Uterine cervix; Immunohistochemistry; Case report
Introduction
Case report
Epithelioid leiomyosarcoma is a rare variant of smooth muscle cell tumor that is histologically characterized by a proliferation of predominantly round and polygonal epithelioid cells with eosinophilic cytoplasm in a malignant tumor component of smooth muscle cells [1]. Although leiomyosarcoma is one of the most common non-epithelial malignant neoplasms arising in soft tissue and somatic organs, cases arising from the uterine cervix are extremely rare. Most epithelioid leiomyosarcomas occur in the uterine body, but only three cases of this tumor originating in the uterine cervix have been reported in the English literature [1–3]. Our objective is to report the clinical and pathological findings of our case and to review the literature of epithelioid leiomyosarcoma arising from the uterine cervix.
A 53-year-old Japanese woman, gravida 2, para 2, was admitted to Tohoku University Hospital from her local hospital with a chief complaint of massive vaginal bleeding. Physical examination revealed a large tumor protruding through the cervical canal with continuous bleeding; the enlarged uterine body was roughly the size of a goose egg. Pelvic magnetic resonance imaging (MRI), smear cytology, and cervical biopsy had already been performed at a previous hospital. Pelvic MRI confirmed the presence of a 7 7 8 cm solid pelvic mass shown to be uterine in origin (Fig. 1). There was no associated lymphadenopathy or other signs of metastatic spread. Smear cytology of the cervical tumor revealed necrotic material and clusters of malignant cells in loose tissue consistent with a tumor (Fig. 2); a cytological diagnosis of malignant tumor cells of possible mesenchymal origin was made. On cervical biopsy, only necrotic tissues were taken and a histological diagnosis
T Corresponding author. Fax: +81 22 717 7258. E-mail address: [email protected] (M. Toyoshima). 0090-8258/$ - see front matter D 2005 Elsevier Inc. All rights reserved. doi:10.1016/j.ygyno.2005.02.028
958
M. Toyoshima et al. / Gynecologic Oncology 97 (2005) 957–960
Fig. 1. A sagittal view of T2-weighted pelvic magnetic resonance image shows a cervical tumor developing towards the vaginal cavity (arrow).
Fig. 3. Gross appearance of the resected specimen. The tumor arising from the uterine cervix is highly necrotic and invades the right vaginal wall (arrowhead). Multiple leiomyomas are seen on the uterine body (arrow).
of a degenerated leiomyoma was determined. Her past medical history was noncontributory. Laboratory studies including lactate dehydrogenase (LDH) and CA-125 antigen levels were within normal limits. Chest X-ray, abdominal X-ray, and electrocardiogram were all normal. On the day she was admitted to our hospital (October 2002), emergency total abdominal hysterectomy and bilateral salpingo-oophorectomy (TAH + BSO) were performed due to massive vaginal bleeding. The tumor arose from the right wall of the uterine cervix and invaded the vagina. Thus, a 3-cm portion of the right vaginal wall was removed with the specimen. The tumor was necrotic and the uterine cervix did not retain its original form. The extracted tumor measured 7 7 8 cm in diameter
(Fig. 3), and the cut surface showed hemorrhage and degenerative changes. Histological examination revealed a predominantly epithelioid neoplasm with focal spindle cells scattered in prominent vascular spaces. Rare foci of hemorrhage and necrosis were present. The cells were small to moderately large with eosinophilic cytoplasm and overall displayed a high nuclear-to-cytoplasmic ratio. The nuclei were highly pleiomorphic and had prominent nucleoli (Figs. 4, 5). The mitotic rate averaged more than 10 mitoses per 10 highpower fields. Immunohistochemical studies showed that the tumor was strongly positive for desmin, smooth muscle actin, and vimentin, findings that are characteristic of
Fig. 2. Smear cytology of the cervical tumor. (A) In the necrotic background, loosely tied cluster of tumor cells is visible. Tumor cells are relatively small and round to polygonal in shape. There are scattered malignant cells around the cluster (100). (B) High-power view of tumor cells shows pleiomorphic nuclei with finely granular chromatin and prominent nucleoli (1000).
Fig. 4. Microscopic view of the tumor lesion. The tumor predominantly consists of round to polygonal cells with significant nuclear atypia in a sheet-like growth pattern (hematoxylin and eosin, 100).
M. Toyoshima et al. / Gynecologic Oncology 97 (2005) 957–960
Fig. 5. High-power view of tumor cells shows highly anaplastic spindle cell proliferation. The tumor cells have enlarged nuclei with unevenly distributed chromatin and prominent nucleoli. Cytoplasm is eosinophilic and mitotic figures are frequently visible (hematoxylin and eosin, 400).
smooth muscle neoplasms. Melan-A staining was only partially positive, and staining for cytokeratin, S-100, and HMB-45 were all negative (Table 1). Following surgery, she was given three courses of ifosfamide 7 mg/m2, doxorubicin 30 mg/m2, and cisplatin 50 mg/m2 (IAP chemotherapy) without radiation therapy. Chest computed tomography (CT) scan performed on 3 months after surgery (January 2003) showed a small nodule in the right lung. The nodule was assumed to represent pulmonary metastases of the primary cervical tumor, and she was switched to different chemotherapeutic regimen, comprising three courses of a combination of paclitaxel 240 mg (175 mg/m2) and carboplatin 500 mg (AUC = 5). A follow-up chest CT scan performed 7 months after surgery (May 2003) detected no metastatic lesions. The patient is now without any evidence of disease on clinical exams and imaging at 20 months after the end of chemotherapy.
Discussion Epithelioid leiomyosarcoma arising from the uterine cervix is an exceedingly rare tumor, with only three
959
previous cases reported in the English literature [1–3]. The clinical courses of these three cases and the present case are summarized in Table 2. The patients’ ages ranged from 47 to 72 years (mean, 58.3). All cases were initially treated by TAH + BSO; two with adjuvant chemotherapy, one with postoperative high-dose vaginal brachytherapy. Two patients developed pulmonary metastases 3 months after surgery despite use of adjuvant chemotherapy. There are two points in common for these four cases: The tumor developed during menopause and there was a tendency to metastasize hematogenously, especially to the lung, much the same pattern as those seen with leiomyosarcomas of the uterine corpus or cervix [4]. These findings support the fact that this type of neoplasm is a variant of leiomyosarcoma, even if it is extremely rare. Because the number of reported cases is too small, no treatment options have been proven to be broadly successful in epithelioid leiomyosarcoma. Considering clinical common-points of comparison between epithelioid and nonepithelioid leiomyosarcoma, consideration of the current accepted standards for management of uterine leiomyosarcoma seems to be most appropriate for weighing management options for epithelioid leiomyosarcoma of the cervix [3]. The standard therapy for uterine leiomyosarcoma has been total abdominal hysterectomy with bilateral salpingooophorectomy. With regard to adjuvant therapy, there is no firm evidence from a prospective study that adjuvant radiation therapy or chemotherapy is of benefit for patients with uterine sarcomas. Several studies show a trend for improved local control in patients receiving pelvic radiation therapy; however, adjuvant radiation therapy does not appear to improve survival for patients with leiomyosarcoma of the uterus [5]. Similarly, adjuvant chemotherapy has not significantly improved survival for patients with uterine leiomyosarcoma. Most patients with advanced disease are considered to be candidates for chemotherapy due to its tendency to metastasize hematogenously. As first line chemotherapy regimen for patients with advanced leiomyosarcoma of the uterus, doxorubicin and ifosfamide show the largest response rates which have been reported as high as 30.3% [6]. For patients with recurrent disease, some responses were reported with a single-agent paclitaxel for leiomyosarcoma [7] and in a combination regimen of paclitaxel and carboplatin for carcinosarcoma [8]. Addition-
Table 1 Summary of immunohistochemical staining results Author
Desmin
SMA
Cytokeratin
S-100
HMB-45
Vimentin
EMA
Melan-A
CD68
Fujiwaki et al. [1] Gotoh et al. [2] Irvin et al. [3] Present case
N – P P
– P P P
Partially P N Partially P N
N N N N
– – N N
P P – P
Partially P N – –
– – – Partially P
– – – N
SMA: smooth muscle protein; EMA: epithelial membrane antigen. –: Not reported, P: positive staining, N: negative staining, partially P: partially positive staining.
960
M. Toyoshima et al. / Gynecologic Oncology 97 (2005) 957–960
Table 2 Summary of epithelioid leiomyosarcomas of the uterine cervix Author
Age
Initial treatment
Adjuvant therapy
Recurrence after initial treatment
States (M)
Fujiwaki et al. [1] Gotoh et al. [2] Irvin et al. [3] Present case
61 72 47 53
TAH TAH TAH TAH
Chemotherapy None Radiation Chemotherapy
Lung metastasis None None Lung metastasis
DOD, 5 NED, 10 NED, 4 NED, 20
+ + + +
BSO BSO BSO BSO
TAH + BSO: total abdominal hysterectomy with salpingo-oophorectomy. DOD: died of disease, NED: no evidence of disease, M: months of follow-up.
ally, the GOG is currently conducting a multi-institutional study of docetaxel and gemcitabine in patients with recurrent or persistent leiomyosarcoma. Morphologically, epithelioid leiomyosarcoma is characterized by sheets and nets of rounded to polygonal cells with abundant eosinophilic cytoplasm and marked cytoplasmic vacuolization [1]. The differential diagnosis for epithelioid leiomyosarcoma on hematoxylin–eosin (H&E) staining, therefore, includes malignant melanoma, metastatic carcinoma, and epithelioid sarcoma. To avoid misdiagnosis of such neoplasms, immunohistochemical features are necessary to support the diagnosis of epithelioid leiomyosarcoma [9]. Typically, melanomas are positive for S-100 protein, HMB-45, Melan-A, and tyrosinase but negative for cytokeratins, desmin, smooth muscle actin, epithelial membrane antigen, and CD68 [10]. Malignant melanoma and metastatic carcinoma are generally excluded due to the immunohistochemical absence of HMB-45 and cytokeratin expression [9]. In contrast to epithelioid leiomyosarcoma, epithelioid sarcoma shows strong and diffuse expression of keratin markers [9]. In addition, desmin positivity is the most specific indicator of smooth muscle differentiation. Immunohistochemical results of previously reported epithelioid leiomyosarcoma cases are summarized in Table 1 with the data for our patient. In summary, because there is so little experience with epithelioid leiomyosarcoma in the uterine cervix, its ultimate prognosis is unclear. Thus, more cases of this unusual morphologic variant and longer follow-up of
existing and future cases are needed to determine the clinical behavior of this neoplasm.
References [1] Fujiwaki R, Yoshida M, Iida K, Ohnishi Y, Ryuko K, Miyazaki K. Epithelioid leiomyosarcoma of the uterine cervix. Acta Obstet Gynecol Scand 1998;77:246 – 8. [2] Gotoh T, Kikuchi Y, Takano M, Kita T, Ogata S, Aida S. Epithelioid leiomyosarcoma of the uterine cervix. Gynecol Oncol 2001;82:400 – 5. [3] Irvin W, Presley A, Andersen W, Taylor P, Rice L. Leiomyosarcoma of the cervix. Gynecol Oncol 2003;91:636 – 42. [4] Abell MR, Ramirez JA. Sarcomas and carcinosarcomas of the uterine cervix. Cancer 1973;31:1176 – 92. [5] Giuntoli RL, Metzinger DS, DiMarco CS, Cha SS, Sloan JA, Keeney GL, et al. Retrospective review of 208 patients with leiomyosarcoma of the uterus: prognostic indicators, surgical management, and adjuvant therapy. Gynecol Oncol 2003;89:460 – 9. [6] Sutton G, Blessing JA, Malfetano JH. Ifosfamide and doxorubicin in the treatment of advanced leiomyosarcomas of the uterus: a gynecologic oncology group study. Gynecol Oncol 1996;62:226 – 9. [7] Gallup DG, Blessing JA, Andersen W, Morgan MA. Evaluation of paclitaxel in previously treated leiomyosarcoma of the uterus: a gynecologic oncology group study. Gynecol Oncol 2003;89:48 – 51. [8] Toyoshima M, Akahira J, Matsunaga G, Niikura H, Ito K, Yaegashi N, et al. Clinical experience with combination paclitaxel and carboplatin therapy for advanced or recurrent carcinosarcoma of the uterus. Gynecol Oncol 2004;94:774 – 8. [9] Yamamoto T, Minami R, Ohbayashi C, Inaba M. Epithelioid leiomyosarcoma of the external deep soft tissue. Arch Pathol Lab Med 2002;126:468 – 70. [10] Banerjee SS, Harris M. Morphological and immunophenotypic variations in malignant melanoma. Histopathology 2000;36:387 – 402.
Case Report
Epithelioid leiomyosarcoma of the uterine cervix: A case report and review of the literature Masafumi ToyoshimaT, Chikako Okamura, Hitoshi Niikura, Kiyoshi Ito, Nobuo Yaegashi Department of Obstetrics and Gynecology, Tohoku University Graduate School of Medicine, 1-1, Seiryo-machi, Aoba, Sendai 980-8574, Japan Received 22 November 2004 Available online 10 May 2005
Abstract Background. Epithelioid leiomyosarcomas arising from the uterine cervix are extremely rare neoplasms, with only three cases reported in the English literature. Case. A 53-year-old Japanese woman was admitted to our university hospital due to massive vaginal bleeding from a cervical tumor. A total hysterectomy with bilateral salpingo-oophorectomy was performed. Histological findings, including immunohistochemical study using desmin, SMA, cytokeratin, S-100, HMB-45, vimentin, melan-A, and CD68, led to a diagnosis of epithelioid leiomyosarcoma of the uterine cervix. The patient underwent adjuvant chemotherapy and has been disease-free for over 20 months. Conclusion. Immunohistochemical studies may be needed to differentiate among the alternative diagnoses of malignant melanoma, metastatic carcinoma, and epithelioid sarcoma. Additional cases are needed to develop optimal management strategies and to predict prognosis. D 2005 Elsevier Inc. All rights reserved. Keywords: Epithelioid leiomyosarcoma; Cervical leiomyosarcoma; Uterine cervix; Immunohistochemistry; Case report
Introduction
Case report
Epithelioid leiomyosarcoma is a rare variant of smooth muscle cell tumor that is histologically characterized by a proliferation of predominantly round and polygonal epithelioid cells with eosinophilic cytoplasm in a malignant tumor component of smooth muscle cells [1]. Although leiomyosarcoma is one of the most common non-epithelial malignant neoplasms arising in soft tissue and somatic organs, cases arising from the uterine cervix are extremely rare. Most epithelioid leiomyosarcomas occur in the uterine body, but only three cases of this tumor originating in the uterine cervix have been reported in the English literature [1–3]. Our objective is to report the clinical and pathological findings of our case and to review the literature of epithelioid leiomyosarcoma arising from the uterine cervix.
A 53-year-old Japanese woman, gravida 2, para 2, was admitted to Tohoku University Hospital from her local hospital with a chief complaint of massive vaginal bleeding. Physical examination revealed a large tumor protruding through the cervical canal with continuous bleeding; the enlarged uterine body was roughly the size of a goose egg. Pelvic magnetic resonance imaging (MRI), smear cytology, and cervical biopsy had already been performed at a previous hospital. Pelvic MRI confirmed the presence of a 7 7 8 cm solid pelvic mass shown to be uterine in origin (Fig. 1). There was no associated lymphadenopathy or other signs of metastatic spread. Smear cytology of the cervical tumor revealed necrotic material and clusters of malignant cells in loose tissue consistent with a tumor (Fig. 2); a cytological diagnosis of malignant tumor cells of possible mesenchymal origin was made. On cervical biopsy, only necrotic tissues were taken and a histological diagnosis
T Corresponding author. Fax: +81 22 717 7258. E-mail address: [email protected] (M. Toyoshima). 0090-8258/$ - see front matter D 2005 Elsevier Inc. All rights reserved. doi:10.1016/j.ygyno.2005.02.028
958
M. Toyoshima et al. / Gynecologic Oncology 97 (2005) 957–960
Fig. 1. A sagittal view of T2-weighted pelvic magnetic resonance image shows a cervical tumor developing towards the vaginal cavity (arrow).
Fig. 3. Gross appearance of the resected specimen. The tumor arising from the uterine cervix is highly necrotic and invades the right vaginal wall (arrowhead). Multiple leiomyomas are seen on the uterine body (arrow).
of a degenerated leiomyoma was determined. Her past medical history was noncontributory. Laboratory studies including lactate dehydrogenase (LDH) and CA-125 antigen levels were within normal limits. Chest X-ray, abdominal X-ray, and electrocardiogram were all normal. On the day she was admitted to our hospital (October 2002), emergency total abdominal hysterectomy and bilateral salpingo-oophorectomy (TAH + BSO) were performed due to massive vaginal bleeding. The tumor arose from the right wall of the uterine cervix and invaded the vagina. Thus, a 3-cm portion of the right vaginal wall was removed with the specimen. The tumor was necrotic and the uterine cervix did not retain its original form. The extracted tumor measured 7 7 8 cm in diameter
(Fig. 3), and the cut surface showed hemorrhage and degenerative changes. Histological examination revealed a predominantly epithelioid neoplasm with focal spindle cells scattered in prominent vascular spaces. Rare foci of hemorrhage and necrosis were present. The cells were small to moderately large with eosinophilic cytoplasm and overall displayed a high nuclear-to-cytoplasmic ratio. The nuclei were highly pleiomorphic and had prominent nucleoli (Figs. 4, 5). The mitotic rate averaged more than 10 mitoses per 10 highpower fields. Immunohistochemical studies showed that the tumor was strongly positive for desmin, smooth muscle actin, and vimentin, findings that are characteristic of
Fig. 2. Smear cytology of the cervical tumor. (A) In the necrotic background, loosely tied cluster of tumor cells is visible. Tumor cells are relatively small and round to polygonal in shape. There are scattered malignant cells around the cluster (100). (B) High-power view of tumor cells shows pleiomorphic nuclei with finely granular chromatin and prominent nucleoli (1000).
Fig. 4. Microscopic view of the tumor lesion. The tumor predominantly consists of round to polygonal cells with significant nuclear atypia in a sheet-like growth pattern (hematoxylin and eosin, 100).
M. Toyoshima et al. / Gynecologic Oncology 97 (2005) 957–960
Fig. 5. High-power view of tumor cells shows highly anaplastic spindle cell proliferation. The tumor cells have enlarged nuclei with unevenly distributed chromatin and prominent nucleoli. Cytoplasm is eosinophilic and mitotic figures are frequently visible (hematoxylin and eosin, 400).
smooth muscle neoplasms. Melan-A staining was only partially positive, and staining for cytokeratin, S-100, and HMB-45 were all negative (Table 1). Following surgery, she was given three courses of ifosfamide 7 mg/m2, doxorubicin 30 mg/m2, and cisplatin 50 mg/m2 (IAP chemotherapy) without radiation therapy. Chest computed tomography (CT) scan performed on 3 months after surgery (January 2003) showed a small nodule in the right lung. The nodule was assumed to represent pulmonary metastases of the primary cervical tumor, and she was switched to different chemotherapeutic regimen, comprising three courses of a combination of paclitaxel 240 mg (175 mg/m2) and carboplatin 500 mg (AUC = 5). A follow-up chest CT scan performed 7 months after surgery (May 2003) detected no metastatic lesions. The patient is now without any evidence of disease on clinical exams and imaging at 20 months after the end of chemotherapy.
Discussion Epithelioid leiomyosarcoma arising from the uterine cervix is an exceedingly rare tumor, with only three
959
previous cases reported in the English literature [1–3]. The clinical courses of these three cases and the present case are summarized in Table 2. The patients’ ages ranged from 47 to 72 years (mean, 58.3). All cases were initially treated by TAH + BSO; two with adjuvant chemotherapy, one with postoperative high-dose vaginal brachytherapy. Two patients developed pulmonary metastases 3 months after surgery despite use of adjuvant chemotherapy. There are two points in common for these four cases: The tumor developed during menopause and there was a tendency to metastasize hematogenously, especially to the lung, much the same pattern as those seen with leiomyosarcomas of the uterine corpus or cervix [4]. These findings support the fact that this type of neoplasm is a variant of leiomyosarcoma, even if it is extremely rare. Because the number of reported cases is too small, no treatment options have been proven to be broadly successful in epithelioid leiomyosarcoma. Considering clinical common-points of comparison between epithelioid and nonepithelioid leiomyosarcoma, consideration of the current accepted standards for management of uterine leiomyosarcoma seems to be most appropriate for weighing management options for epithelioid leiomyosarcoma of the cervix [3]. The standard therapy for uterine leiomyosarcoma has been total abdominal hysterectomy with bilateral salpingooophorectomy. With regard to adjuvant therapy, there is no firm evidence from a prospective study that adjuvant radiation therapy or chemotherapy is of benefit for patients with uterine sarcomas. Several studies show a trend for improved local control in patients receiving pelvic radiation therapy; however, adjuvant radiation therapy does not appear to improve survival for patients with leiomyosarcoma of the uterus [5]. Similarly, adjuvant chemotherapy has not significantly improved survival for patients with uterine leiomyosarcoma. Most patients with advanced disease are considered to be candidates for chemotherapy due to its tendency to metastasize hematogenously. As first line chemotherapy regimen for patients with advanced leiomyosarcoma of the uterus, doxorubicin and ifosfamide show the largest response rates which have been reported as high as 30.3% [6]. For patients with recurrent disease, some responses were reported with a single-agent paclitaxel for leiomyosarcoma [7] and in a combination regimen of paclitaxel and carboplatin for carcinosarcoma [8]. Addition-
Table 1 Summary of immunohistochemical staining results Author
Desmin
SMA
Cytokeratin
S-100
HMB-45
Vimentin
EMA
Melan-A
CD68
Fujiwaki et al. [1] Gotoh et al. [2] Irvin et al. [3] Present case
N – P P
– P P P
Partially P N Partially P N
N N N N
– – N N
P P – P
Partially P N – –
– – – Partially P
– – – N
SMA: smooth muscle protein; EMA: epithelial membrane antigen. –: Not reported, P: positive staining, N: negative staining, partially P: partially positive staining.
960
M. Toyoshima et al. / Gynecologic Oncology 97 (2005) 957–960
Table 2 Summary of epithelioid leiomyosarcomas of the uterine cervix Author
Age
Initial treatment
Adjuvant therapy
Recurrence after initial treatment
States (M)
Fujiwaki et al. [1] Gotoh et al. [2] Irvin et al. [3] Present case
61 72 47 53
TAH TAH TAH TAH
Chemotherapy None Radiation Chemotherapy
Lung metastasis None None Lung metastasis
DOD, 5 NED, 10 NED, 4 NED, 20
+ + + +
BSO BSO BSO BSO
TAH + BSO: total abdominal hysterectomy with salpingo-oophorectomy. DOD: died of disease, NED: no evidence of disease, M: months of follow-up.
ally, the GOG is currently conducting a multi-institutional study of docetaxel and gemcitabine in patients with recurrent or persistent leiomyosarcoma. Morphologically, epithelioid leiomyosarcoma is characterized by sheets and nets of rounded to polygonal cells with abundant eosinophilic cytoplasm and marked cytoplasmic vacuolization [1]. The differential diagnosis for epithelioid leiomyosarcoma on hematoxylin–eosin (H&E) staining, therefore, includes malignant melanoma, metastatic carcinoma, and epithelioid sarcoma. To avoid misdiagnosis of such neoplasms, immunohistochemical features are necessary to support the diagnosis of epithelioid leiomyosarcoma [9]. Typically, melanomas are positive for S-100 protein, HMB-45, Melan-A, and tyrosinase but negative for cytokeratins, desmin, smooth muscle actin, epithelial membrane antigen, and CD68 [10]. Malignant melanoma and metastatic carcinoma are generally excluded due to the immunohistochemical absence of HMB-45 and cytokeratin expression [9]. In contrast to epithelioid leiomyosarcoma, epithelioid sarcoma shows strong and diffuse expression of keratin markers [9]. In addition, desmin positivity is the most specific indicator of smooth muscle differentiation. Immunohistochemical results of previously reported epithelioid leiomyosarcoma cases are summarized in Table 1 with the data for our patient. In summary, because there is so little experience with epithelioid leiomyosarcoma in the uterine cervix, its ultimate prognosis is unclear. Thus, more cases of this unusual morphologic variant and longer follow-up of
existing and future cases are needed to determine the clinical behavior of this neoplasm.
References [1] Fujiwaki R, Yoshida M, Iida K, Ohnishi Y, Ryuko K, Miyazaki K. Epithelioid leiomyosarcoma of the uterine cervix. Acta Obstet Gynecol Scand 1998;77:246 – 8. [2] Gotoh T, Kikuchi Y, Takano M, Kita T, Ogata S, Aida S. Epithelioid leiomyosarcoma of the uterine cervix. Gynecol Oncol 2001;82:400 – 5. [3] Irvin W, Presley A, Andersen W, Taylor P, Rice L. Leiomyosarcoma of the cervix. Gynecol Oncol 2003;91:636 – 42. [4] Abell MR, Ramirez JA. Sarcomas and carcinosarcomas of the uterine cervix. Cancer 1973;31:1176 – 92. [5] Giuntoli RL, Metzinger DS, DiMarco CS, Cha SS, Sloan JA, Keeney GL, et al. Retrospective review of 208 patients with leiomyosarcoma of the uterus: prognostic indicators, surgical management, and adjuvant therapy. Gynecol Oncol 2003;89:460 – 9. [6] Sutton G, Blessing JA, Malfetano JH. Ifosfamide and doxorubicin in the treatment of advanced leiomyosarcomas of the uterus: a gynecologic oncology group study. Gynecol Oncol 1996;62:226 – 9. [7] Gallup DG, Blessing JA, Andersen W, Morgan MA. Evaluation of paclitaxel in previously treated leiomyosarcoma of the uterus: a gynecologic oncology group study. Gynecol Oncol 2003;89:48 – 51. [8] Toyoshima M, Akahira J, Matsunaga G, Niikura H, Ito K, Yaegashi N, et al. Clinical experience with combination paclitaxel and carboplatin therapy for advanced or recurrent carcinosarcoma of the uterus. Gynecol Oncol 2004;94:774 – 8. [9] Yamamoto T, Minami R, Ohbayashi C, Inaba M. Epithelioid leiomyosarcoma of the external deep soft tissue. Arch Pathol Lab Med 2002;126:468 – 70. [10] Banerjee SS, Harris M. Morphological and immunophenotypic variations in malignant melanoma. Histopathology 2000;36:387 – 402.