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Primary Liposarcoma of the Cervix: A Case Report Andrew J. Veiiath, P. Hannah, C. Ratnakar, K. Jayanthi and Amrit L. Aurora Department of Pathology, Jawaharlal Institute oj Postfiiaduate Medical Education and Research, Pondicherry. India Department nf Obstetrics and Gynecology, Church of South India Hospital. Xenoor, India
ABSTRACT Veiiath AJ, Hannah P, Ratnakar C, Jayanthi K, Aurora AL (Dept of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research, Pondkherry— 605006, and Dept of Obstetrics and Gynecology, Church of South India Hospital, Neyyoor, India). Primary liposarcoma of the cervix: a case report. hit J Gynaecol Obstet 16: 75-79, 1978 A case of primary liposarcoma of the cervix is described in a 59-year-old postmenopausal female who was diagnosed clinically as having a cervical fibroid. Pathologic features and the probable histogenesis are discussed. Primary liposarcoma of the cervix does not appear to have been described in the literature.
INTRODUCTION Sarcomas arising from the uterine corpus are well documented (4, 7, 8, 10). Cervical sarcomas, however, are uncommon and may be either pure lesions consisting of a uniform cell type or mixed mesodermal tumors (6). T h e majority of pure sarcomas of the cervix are malignant tumors of smooth muscle origin (6, 9). There are very few descriptions in the literature of cervical sarcomas which originate in other cell types. We describe a polypoidal tumor of the cervix which proved to be a pure liposarcoma. To our knowledge, this is the first description of primary liposarcoma of the cervix.
CASE R E P O R T A 59-year-old woman was admitted to our hospital on J u n e 16, 1977, complaining of a bloodstained vaginal discharge of 15 days' duration. The patient had been married for 35 years and had five full-term normal deliveries. Four children were alive and healthy; the youngest child was 21
years old. The patient had reached menopause at the age of 54 and since then had been free of genital tract symptoms until 15 days before this admission. She was a known hypertensive of some years' duration, and her blood pressure (BP) was controlled by a daily intake of reserpine. The patient was well nourished and did not appear anemic. Her pulse rate was 90/min and her blood pressure was 120/90 mm Hg after reserpine. Her peripheral lymph nodes were not enlarged. Examination of all systems other than the genital tract revealed no abnormality. Vaginal examination revealed a soft-to-firm polypoidal mass replacing the posterior lip of the cervix, which was in midposition. The cervical rim was thinned out and palpable on all but the posterior side. The uterus was slightly enlarged and in midposition; the fornices appeared free. Laboratory investigations showed a total leukocyte count of 8 2 0 0 / m m ' , with a differential count of 44% neutrophils and 56% lymphocytes. The hemoglobin level was 10.5 gm/100 ml. Urinalysis revealed only a trace of albumin and a few epithelial cells. Ova of Ascaris lumbncoides and Ancylostoma duodenale were demonstrated in the stool and were treated successfully. Blood sugar and urea estimations were normal. A chest x-ray and electrocardiogram were normal. The patient was thought to have cervical fibroids, and other uterine fibroids were suspected. A total abdominal hysterectomy with bilateral salpingo-oophorectomy was performed on J u n e 29, 1977. At operation, the entire posterior wall of the cervix was found to be replaced by a polypoidal tumor which was partially adherent to the broad ligament on the left. The uterine corpus was unremarkable. T h e immediate postoperative period was uneventful. On August 6, 1977, the patient complained of a blood-stained vaginal discharge. On examination, a small, partially hemorrhagic tumor nodule was seen in the vaginal wall. T h e tumor was excised and the patient was started on radiotherapy.
InlJ Gynaecol Obstet 16
76
I 'eliath el al
Pathologic features Gross. T h e uterus and cervix measured 9.5 cm X 6.0 cm X 3.0 cm and showed a well-circumscribed polypoidal tumor replacing the entire posterior wall of the cervix (Fig. 1). The tumor was not encapsulated, was yellowish white with a soft-to-firm consistency and measured 5 cm X 3 cm. The cut section of the tumor revealed its interior to be yellowish white throughout with hemorrhagic and necrotic foci (Fig. 2). There was no evidence of infiltration of the uterine musculature. The endometrium was unremarkable, except for a polyp measuring 2.0 cm X 1.0 cm X 0.5 cm arising from the posterior surface (Figs. 1 and 2). The myometrium was of normal thickness and showed a single intramural leiomyoma measuring 2 cm in diameter situated in the anterior wall. There were no subserous or submucous leiomyomas. The right and left ovary and tube were normal. Microscopy. Microscopic examination of multiple sections from different areas of the cervical tumor and vaginal nodule showed a uniform histologic pattern without a variation in the cell type or presence of other tissue elements. The tumor consisted of round to polyhedral cells with a pale eosinophilic vacuolated cytoplasm and poorly defined cell outlines (Fig. 3). The nuclei were typically
vesicular and many cells displayed a prominent nucleolus. Some cells had a hyperchromatic nucleus with clumped chromatin. There were scattered giant cells with multilobed hyperchromatic nuclei and vacuolated cytoplasm. Mitotic counts ranged from one to two mitoses in each high-power field, with occasional abnormal mitoses. Special staining procedures for mucin, periodic acid-Schiff positivitv and cross-striations were negative. Rcticulin stains showed rcticulin fibers around individual cells or small clumps. Frozen sections of tumor taken from multiple areas without foci of necrosis or hemorrhage and stained with oil red 0, Sudan Black B and Sudan IV were strongly positive and revealed a lipid content in almost every cell (Fig. 4). T h e endometrial polyp showed a Swiss-cheese pattern, with many cystically dilated glands lined by columnar epithelium. There was no evidence of ulceration or hemorrhage. The intramural leiomyoma showed a typical structure with interlacing spindle-shaped cells. Sections taken from the rest of the uterus and from both ovaries and tubes were unremarkable. DISCUSSION Lipomatous tumors of the cervix are extremely rare. A literature review shows that only benign
4270/77f
Fig. 1. Panhysterectomy specimen with the uterus opened anteriorly showing a polypoidal tumor of the cervix and an endometrial polyp in the fundus.
¡nl J Gynaecol Obstei ¡6
I.ip(isiira>ma uj the cervix
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j| 4 2 7 0 / 7 7 1 1 1 1 1 1 1 1 1 1 1 1 1 1
» i » 1 1 1 1 • 11 • ~ i
11
56
111 » i • ' • • i • •
Fig. 2. Cut surface of the cervical tumor and uterus. The tumor is homogeneous and well circumscribed without infiltration of the uterine wall and shows an area of hemorrhage. The endometrial polyp is at the lower right.
Fig. 3. Section from the tumor showing marked cytoplasmic vacuolation of the tumor cells. Giant cells with hyperchromatic multilobed nuclei and a mitotic figure are evident (HE, x 6 6 6 ) .
In! J Cynaecol Obstei 16
78
Velialh el al
•Mi
Fig. 4 . Frozen section of cervical tumor stained for lipid. Intracellular lipid content of the tumor cells is marked (Sudan Black B, X 6 6 6 ) .
lesions designated as pure or mixed lipomas have been observed in the cervix and that such observations have been rare (2). Lipomas of the uterine corpus are, however, well documented (2, 3, 6). To our knowledge, primary liposarcoma of the cervix has not been described. T h e cervical tumor in the case reported was diagnosed as liposarcoma on the basis of cytologic features and the marked intracellular lipid. T h e striking fat positivity of tissues from multiple areas of the tumor ruled out the possibility of cell degeneration and secondary fat accumulation. The lipid content and uniformity of cell type suggested against other lesions which were considered as possibilities in the differential diagnosis. Bizarre or epithelioid leiomyoma (10) and epithelioid leiomyosarcoma (1) show certain microscopic structural similarities with the reported tumor, but neither contains intracellular lipid. Mixed mesodermal tumors of the uterus may show foci of liposarcoma (4). Detailed examination of these tumors, however, always reveals an admixture of different tissue elements. T h e cytologic features of the reported tumor suggested a classification as round cell liposarcoma (5). Kindblom et al (5), in a study of 122 cases of
Int J Gynaecol Obstet 16
liposarcoma, observed that the round cell variety showed an average age of onset of 6Ü years and was usually located in the retroperitoneum, lower extremities or in the trunk and neck. Liposarcomas of the uterus and cervix were not seen by Kindblom et al (5). Ober and Tovell (8), in a study of mesenchymal sarcomas of the uterus, observed that the pure heterologous tumors usually occurred in postmenopausal women, metastasized widely and had a poor prognosis. It is of interest that the patient reported was a 59-year-old postmenopausal woman in whom a metastatic tumor was seen in the vagina less than two months following total abdominal hysterectomy. O n e theory suggests that uterine lipomas originate from metaplasia of smooth muscle cells in the uterine wall or in leiomyomas (2, 3, 6, 7). T h e observed coexistence of leiomyomas with uterine lipomas (3) suggests the latter process as being the more frequent. It is theoretically possible for a liposarcoma to arise in a preexisting leiomyoma by metaplasia and malignant transformation. In support of such a possibility are the reports of Scheffey et al (11) and Stier and Lyman (12), which describe cases of primary osteosarcoma of the uterus origi-
I.ifmsuraima iij the cervix
nating in a preexisting leiomyoma. These observations, together with the well-circumscribed configuration of the cervical tumor and the associated uterine leiomyoma in our case, suggest that the liposarcoma originates in a preexisting cervical leiomyoma.
7. 8. 9. 10.
REFERENCES 1 1. 1. Appelman H D , Helwig EB: Sarcomas of the stomach. Am J Clin Pathol 1,7:2, 1977. 2. Brandfass R T , Everts-Suarez EA: Lipomatous tumors of the uterus: a review of the world's literature with report of a case of true lipoma. Am J Obstet Gynecol 7(7:359. 1955. 3. Gonzalez-Angulo A, Kaufman R H : Lipomatous tumor of the uterus: report of a case. Obstet Gynecol /.'7'494. 1962. 4. Kempson RL: Sarcomas and related neoplasms. In T h e Uterus (ed HJ Norris, A T Hertig. M R Abell), p 298. Williams & VVilkins Co, Baltimore, 1973. 5. Kindblom LG, Angervall L, Svendsen P: Liposarcoma: a clinicopathologic, radiographic and prognostic study. Acta Pathol Microbiol Scand |A) Suppl 253. 1975. 6. Novak ER, Woodruff J D : Novak's Gynecologic and O b -
12.
79
stetric Pathology: With Clinical and Endocrine Relations, 7th ed. WB Saunders Co, Philadelphia. 1974. Ober W B : Uterine sarcomas: histogenesis and taxonomv. Ann NY Acad Sei 7.7:568. 1959. Ober WB, Tovell H M M : Mesenchymal sarcomas of the uterus. Am J Obstet Gynecol 77:246. 1959. Rothbard MJ, M a r k h a m E H : Leiomyosarcoma of the cervix: report of a case. Am J Obstet Gynecol /_ ; 0:853. 1974. Saksela E. Lampinen Y, Procopé BJ: Malignant mesenchymal tumors of the uterine corpus. Am J Obstet Gvnecol 12
Primary Liposarcoma of the Cervix: A Case Report Andrew J. Veiiath, P. Hannah, C. Ratnakar, K. Jayanthi and Amrit L. Aurora Department of Pathology, Jawaharlal Institute oj Postfiiaduate Medical Education and Research, Pondicherry. India Department nf Obstetrics and Gynecology, Church of South India Hospital. Xenoor, India
ABSTRACT Veiiath AJ, Hannah P, Ratnakar C, Jayanthi K, Aurora AL (Dept of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research, Pondkherry— 605006, and Dept of Obstetrics and Gynecology, Church of South India Hospital, Neyyoor, India). Primary liposarcoma of the cervix: a case report. hit J Gynaecol Obstet 16: 75-79, 1978 A case of primary liposarcoma of the cervix is described in a 59-year-old postmenopausal female who was diagnosed clinically as having a cervical fibroid. Pathologic features and the probable histogenesis are discussed. Primary liposarcoma of the cervix does not appear to have been described in the literature.
INTRODUCTION Sarcomas arising from the uterine corpus are well documented (4, 7, 8, 10). Cervical sarcomas, however, are uncommon and may be either pure lesions consisting of a uniform cell type or mixed mesodermal tumors (6). T h e majority of pure sarcomas of the cervix are malignant tumors of smooth muscle origin (6, 9). There are very few descriptions in the literature of cervical sarcomas which originate in other cell types. We describe a polypoidal tumor of the cervix which proved to be a pure liposarcoma. To our knowledge, this is the first description of primary liposarcoma of the cervix.
CASE R E P O R T A 59-year-old woman was admitted to our hospital on J u n e 16, 1977, complaining of a bloodstained vaginal discharge of 15 days' duration. The patient had been married for 35 years and had five full-term normal deliveries. Four children were alive and healthy; the youngest child was 21
years old. The patient had reached menopause at the age of 54 and since then had been free of genital tract symptoms until 15 days before this admission. She was a known hypertensive of some years' duration, and her blood pressure (BP) was controlled by a daily intake of reserpine. The patient was well nourished and did not appear anemic. Her pulse rate was 90/min and her blood pressure was 120/90 mm Hg after reserpine. Her peripheral lymph nodes were not enlarged. Examination of all systems other than the genital tract revealed no abnormality. Vaginal examination revealed a soft-to-firm polypoidal mass replacing the posterior lip of the cervix, which was in midposition. The cervical rim was thinned out and palpable on all but the posterior side. The uterus was slightly enlarged and in midposition; the fornices appeared free. Laboratory investigations showed a total leukocyte count of 8 2 0 0 / m m ' , with a differential count of 44% neutrophils and 56% lymphocytes. The hemoglobin level was 10.5 gm/100 ml. Urinalysis revealed only a trace of albumin and a few epithelial cells. Ova of Ascaris lumbncoides and Ancylostoma duodenale were demonstrated in the stool and were treated successfully. Blood sugar and urea estimations were normal. A chest x-ray and electrocardiogram were normal. The patient was thought to have cervical fibroids, and other uterine fibroids were suspected. A total abdominal hysterectomy with bilateral salpingo-oophorectomy was performed on J u n e 29, 1977. At operation, the entire posterior wall of the cervix was found to be replaced by a polypoidal tumor which was partially adherent to the broad ligament on the left. The uterine corpus was unremarkable. T h e immediate postoperative period was uneventful. On August 6, 1977, the patient complained of a blood-stained vaginal discharge. On examination, a small, partially hemorrhagic tumor nodule was seen in the vaginal wall. T h e tumor was excised and the patient was started on radiotherapy.
InlJ Gynaecol Obstet 16
76
I 'eliath el al
Pathologic features Gross. T h e uterus and cervix measured 9.5 cm X 6.0 cm X 3.0 cm and showed a well-circumscribed polypoidal tumor replacing the entire posterior wall of the cervix (Fig. 1). The tumor was not encapsulated, was yellowish white with a soft-to-firm consistency and measured 5 cm X 3 cm. The cut section of the tumor revealed its interior to be yellowish white throughout with hemorrhagic and necrotic foci (Fig. 2). There was no evidence of infiltration of the uterine musculature. The endometrium was unremarkable, except for a polyp measuring 2.0 cm X 1.0 cm X 0.5 cm arising from the posterior surface (Figs. 1 and 2). The myometrium was of normal thickness and showed a single intramural leiomyoma measuring 2 cm in diameter situated in the anterior wall. There were no subserous or submucous leiomyomas. The right and left ovary and tube were normal. Microscopy. Microscopic examination of multiple sections from different areas of the cervical tumor and vaginal nodule showed a uniform histologic pattern without a variation in the cell type or presence of other tissue elements. The tumor consisted of round to polyhedral cells with a pale eosinophilic vacuolated cytoplasm and poorly defined cell outlines (Fig. 3). The nuclei were typically
vesicular and many cells displayed a prominent nucleolus. Some cells had a hyperchromatic nucleus with clumped chromatin. There were scattered giant cells with multilobed hyperchromatic nuclei and vacuolated cytoplasm. Mitotic counts ranged from one to two mitoses in each high-power field, with occasional abnormal mitoses. Special staining procedures for mucin, periodic acid-Schiff positivitv and cross-striations were negative. Rcticulin stains showed rcticulin fibers around individual cells or small clumps. Frozen sections of tumor taken from multiple areas without foci of necrosis or hemorrhage and stained with oil red 0, Sudan Black B and Sudan IV were strongly positive and revealed a lipid content in almost every cell (Fig. 4). T h e endometrial polyp showed a Swiss-cheese pattern, with many cystically dilated glands lined by columnar epithelium. There was no evidence of ulceration or hemorrhage. The intramural leiomyoma showed a typical structure with interlacing spindle-shaped cells. Sections taken from the rest of the uterus and from both ovaries and tubes were unremarkable. DISCUSSION Lipomatous tumors of the cervix are extremely rare. A literature review shows that only benign
4270/77f
Fig. 1. Panhysterectomy specimen with the uterus opened anteriorly showing a polypoidal tumor of the cervix and an endometrial polyp in the fundus.
¡nl J Gynaecol Obstei ¡6
I.ip(isiira>ma uj the cervix
O
j| 4 2 7 0 / 7 7 1 1 1 1 1 1 1 1 1 1 1 1 1 1
» i » 1 1 1 1 • 11 • ~ i
11
56
111 » i • ' • • i • •
Fig. 2. Cut surface of the cervical tumor and uterus. The tumor is homogeneous and well circumscribed without infiltration of the uterine wall and shows an area of hemorrhage. The endometrial polyp is at the lower right.
Fig. 3. Section from the tumor showing marked cytoplasmic vacuolation of the tumor cells. Giant cells with hyperchromatic multilobed nuclei and a mitotic figure are evident (HE, x 6 6 6 ) .
In! J Cynaecol Obstei 16
78
Velialh el al
•Mi
Fig. 4 . Frozen section of cervical tumor stained for lipid. Intracellular lipid content of the tumor cells is marked (Sudan Black B, X 6 6 6 ) .
lesions designated as pure or mixed lipomas have been observed in the cervix and that such observations have been rare (2). Lipomas of the uterine corpus are, however, well documented (2, 3, 6). To our knowledge, primary liposarcoma of the cervix has not been described. T h e cervical tumor in the case reported was diagnosed as liposarcoma on the basis of cytologic features and the marked intracellular lipid. T h e striking fat positivity of tissues from multiple areas of the tumor ruled out the possibility of cell degeneration and secondary fat accumulation. The lipid content and uniformity of cell type suggested against other lesions which were considered as possibilities in the differential diagnosis. Bizarre or epithelioid leiomyoma (10) and epithelioid leiomyosarcoma (1) show certain microscopic structural similarities with the reported tumor, but neither contains intracellular lipid. Mixed mesodermal tumors of the uterus may show foci of liposarcoma (4). Detailed examination of these tumors, however, always reveals an admixture of different tissue elements. T h e cytologic features of the reported tumor suggested a classification as round cell liposarcoma (5). Kindblom et al (5), in a study of 122 cases of
Int J Gynaecol Obstet 16
liposarcoma, observed that the round cell variety showed an average age of onset of 6Ü years and was usually located in the retroperitoneum, lower extremities or in the trunk and neck. Liposarcomas of the uterus and cervix were not seen by Kindblom et al (5). Ober and Tovell (8), in a study of mesenchymal sarcomas of the uterus, observed that the pure heterologous tumors usually occurred in postmenopausal women, metastasized widely and had a poor prognosis. It is of interest that the patient reported was a 59-year-old postmenopausal woman in whom a metastatic tumor was seen in the vagina less than two months following total abdominal hysterectomy. O n e theory suggests that uterine lipomas originate from metaplasia of smooth muscle cells in the uterine wall or in leiomyomas (2, 3, 6, 7). T h e observed coexistence of leiomyomas with uterine lipomas (3) suggests the latter process as being the more frequent. It is theoretically possible for a liposarcoma to arise in a preexisting leiomyoma by metaplasia and malignant transformation. In support of such a possibility are the reports of Scheffey et al (11) and Stier and Lyman (12), which describe cases of primary osteosarcoma of the uterus origi-
I.ifmsuraima iij the cervix
nating in a preexisting leiomyoma. These observations, together with the well-circumscribed configuration of the cervical tumor and the associated uterine leiomyoma in our case, suggest that the liposarcoma originates in a preexisting cervical leiomyoma.
7. 8. 9. 10.
REFERENCES 1 1. 1. Appelman H D , Helwig EB: Sarcomas of the stomach. Am J Clin Pathol 1,7:2, 1977. 2. Brandfass R T , Everts-Suarez EA: Lipomatous tumors of the uterus: a review of the world's literature with report of a case of true lipoma. Am J Obstet Gynecol 7(7:359. 1955. 3. Gonzalez-Angulo A, Kaufman R H : Lipomatous tumor of the uterus: report of a case. Obstet Gynecol /.'7'494. 1962. 4. Kempson RL: Sarcomas and related neoplasms. In T h e Uterus (ed HJ Norris, A T Hertig. M R Abell), p 298. Williams & VVilkins Co, Baltimore, 1973. 5. Kindblom LG, Angervall L, Svendsen P: Liposarcoma: a clinicopathologic, radiographic and prognostic study. Acta Pathol Microbiol Scand |A) Suppl 253. 1975. 6. Novak ER, Woodruff J D : Novak's Gynecologic and O b -
12.
79
stetric Pathology: With Clinical and Endocrine Relations, 7th ed. WB Saunders Co, Philadelphia. 1974. Ober W B : Uterine sarcomas: histogenesis and taxonomv. Ann NY Acad Sei 7.7:568. 1959. Ober WB, Tovell H M M : Mesenchymal sarcomas of the uterus. Am J Obstet Gynecol 77:246. 1959. Rothbard MJ, M a r k h a m E H : Leiomyosarcoma of the cervix: report of a case. Am J Obstet Gynecol /_ ; 0:853. 1974. Saksela E. Lampinen Y, Procopé BJ: Malignant mesenchymal tumors of the uterine corpus. Am J Obstet Gvnecol 12
