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Sclerosing stromal tumor of the ovary in a 4-year-old girl with characteristics of an ovarian signet-ring stromal tumor
ARTICLE IN PRESS Pathology – Research and Practice 206 (2010) 338–341
Contents lists available at ScienceDirect
Pathology – Research and Practice journal homepage: www.elsevier.de/prp
Teaching case
Sclerosing stromal tumor of the ovary in a 4-year-old girl with characteristics of an ovarian signet-ring stromal tumor Ying He a, Kai-xuan Yang a,, Wei Jiang a, Dan-qing Wang b, Lei Li a a b
Department of Pathology, West China Second Hospital of Sichuan University, Chengdu, People’s Republic of China Departments of Obstetrics and Gynecology, West China Second Hospital of Sichuan University, The People of South Road 20, Chengdu 610041, People’s Republic of China
a r t i c l e in f o
a b s t r a c t
Article history: Received 18 March 2009 Received in revised form 27 April 2009 Accepted 11 June 2009
Ovarian sclerosing stromal tumor (OSST) is an extremely rare neoplasm that primarily affects young women. Signet-ring stromal tumor is another rare non-functioning benign ovarian stromal neoplasm. We report a case of a right OSST with prominent characteristics of signet-ring stromal tumor in a 4-yearold girl with symptoms of premature thelarche. We describe the clinical, histopathological, and immunohistochemical findings and review the literature. To our knowledge, the 4-year-old patient presented here is the youngest case of OSST reported in premenarchal children. The presence of nonmucin/non-lipid obvious signet-ring-like cells in this case suggests a possible relationship between OSST and signet-ring stromal tumor of the ovary. Crown Copyright & 2009 Published by Elsevier GmbH. All rights reserved.
Keywords: Sclerosing stromal tumor Signet-ring stromal tumor Ovary
Introduction Ovarian sclerosing stromal tumor (OSST), a rare subtype of benign ovarian neoplasm derived from the sex-cord stroma, was first identified by Chalvardjian and Scully in 1973 [1]. It tends to occur in the second to third decade of life. Only three cases have been reported in adolescent girls, as early as 10 years old [2–4]. The youngest case of OSST ever documented is a 7-month-old infant with symptoms of vaginal bleeding at presentation [5]. We describe the case of an OSST excised from a 4-year-old girl, the youngest child patient reported in the English literature. Signet-ring stromal tumor of the ovary is another extremely rare non-functioning distinctive stromal neoplasm that was first described by Ramzy in 1976 [6], with only 11 cases previously reported in the literature [7]. It occurs in adults, affecting patients between 21 and 83 years of age [8].
Clinical history A 4-year-old premenarchal girl was admitted to our hospital because of premature thelarche as diagnosed by her child health doctor. Her previous medical history and her family history were unremarkable. The results of the laboratory studies were as follows: estradiol levels, 37.2 pg/ml; progesterone levels, 0.25 ng/ ml; testosterone levels, 0.07 ng/ml; human chorionic gonadotropin levels, 0 mIU/ml; follicle-stimulating hormone, o1.0 mIU/ml; Corresponding author. Tel.: +86 028 85503177; fax: +86 028 85501359.
E-mail address: [email protected] (K.-x. Yang).
luteinizing hormone, o1.0 mIU/ml; a-fetoprotein, 3.1 ng/ml; prolactin, 4.8 ng/ml. Ultrasonography and computed tomography of the abdomen and pelvic revealed a right ovarian mass approximately 4 cm in diameter with cystic-like degeneration. Under the pre-operative diagnosis of right ovarian tumor, the patient underwent exploratory laparotomy. A smooth mass with welldefined margins, measuring 4.4 3.6 3.4 cm, was found to arise within the right ovary, and was resected. The patient was free of disease, with a follow-up of 4 months.
Materials and methods Tissue was fixed in 10% buffered formalin and embedded in paraffin. Four-micrometer sections were stained with hematoxylin and eosin, as well as with mucicarmine (mucin), PAS with and without diastase digestion, and Alcian blue (AB). For lipid (SudanIII) staining, sections were obtained from frozen tissue. Immunohistochemistry was performed using the avidin–biotin–peroxidase complex method. The antibodies used included vimentin (Dakopatts, Glostrup, Denmark), smooth muscle actin( SMA) (Dako), CD99 (Dako), calretinin (Dako), CD56 (Dako), a-inhibin (Dako), S-100 (Dako), estrogen receptors (ER) (Immunotech), progesterone receptors (PR) (Immunotech), cytokeratins (Dako), and MIB-1 (Zymed, South San Francisco, USA).
Results Macroscopically, the tumor presented as a well-demarcated lump. Sectioning disclosed that tumor was solid, and contained a
0344-0338/$ - see front matter Crown Copyright & 2009 Published by Elsevier GmbH. All rights reserved. doi:10.1016/j.prp.2009.06.005
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Fig. 1. (A) Nodular architecture of tumor (H&E 100). (B) Obvious collagenous areas (H&E 400). (C) Hemangiopericytoma-like vasculature (H&E 200). (D) The signetring-like cells possess eccentric nuclei and clear cytoplasm containing single large vacuoles (H&E 400) (indicated by arrows). (E). Cellular nodules transform into hypocellular and hyalinized structures (H&E 200). (F) Signet-ring-like cells are admixed with spindle-shaped cells outside the nodules (H&E 400) (indicated by arrows).
unilocular cyst measuring 1.5 1.0 1.0 cm. The solid component consisted of white-to-pale yellow whorled tissue. No hemorrhagic or necrotic areas were noted. Microscopically, the tumors showed a pseudolobular growth pattern with cellular nodules (Fig. 1A) separated by spindleshaped, fibroblast-like cells, hypocellular areas, collagenous areas (Fig. 1B), and hemangiopericytomatous vasculature (Fig. 1C). The cellular nodules varied in size, and some cellular nodules were primarily composed of round and epithelioid cells containing eccentric nuclei and abundant vacuolated cytoplasm resembling signet-ring cells (Fig. 1D), which eventually transformed into hypocellular and hyalinized structure (Fig. 1E). Signet-ring cells were also admixed with spindle-shaped stromal cells outside the nodules (Fig. 1F). Mitoses were extremely rare. Immunohistochemically, clear vacuoles of the signet-ring-like cells were negative for PAS with and without diastase digestion, lipids, mucin, and AB stains. The signet-ring cells showed diffusely positive immunoreactivity for vimentin (Fig. 2A), SMA, CD99, calretinin (Fig. 2B), CD56 (Fig. 2C), focal staining for a-inhibin (Fig. 2D), S-100 proteins, and PR (Fig. 2E). They were negative for ER and cytokeratins. The fibroblast-like cells only had positivity for Vimentin and were weakly patchy-positive for calretinin. MIB-1 (Ki 67) immunohistochemistry showed a positive reaction in about 10% of the nuclei of the round cells (Fig. 2F).
Discussion Ovarian sclerosing stromal tumor is a rare benign ovarian neoplasm derived from a population of muscle-specific, actinpositive elements from the perifollicular myoid stromal cell [9]. It is usually unilateral, although bilateral cases were reported by Chang et al. [2]. A particularly interesting case of SST arising in an accessory ovary has also been described [10]. Only three cases of OSST have been reported in adolescent girls. The main clinico-
pathologic features of those reports are summarized in Table 1. In our case, positivity for CD99, calretinin, CD56, and a-inhibin in the round tumor cells demonstrated an ovarian stromal origin of the neoplasm. This tumor was characterized by a pseudolobular growth pattern, cellular nodules separated by spindle-shaped stromal cells, varying degrees of collagenization, and hemangiopericytoma-like vasculature. The architecture of cellular nodules found in this case, gradually undergoing loss of cells, leading to hypocellular and hyalinized nodules, was stressed only by Hall et al. [5] and not emphasized in other reports. In the original description, the authors did not mention any hormonal activity of the tumor. However, evidence of hormonal activity has been reported in subsequent cases, showing increased levels of either estrogen, progesterone, or androgens as well as clinical symptoms, including menstrual cycle disturbances, hirsutism, masculinization, precocious puberty, and vaginal bleeding [5]. The cells of OSST were enzymatically active for estradiol 17-bdehydrogenase, glucose-6-phosphate dehydrogenase, and isoligandin citric dehydrogenase [11]. This result showed that the tumor cells can synthesize dehydroepiandrosterone and estradiol from circulating estrone. The signet-ring stromal tumors of the ovary reported so far were hormonally inactive, although the progesterone receptors were found to be strongly and uniformly positively expressed [7]. In this case, the girl was admitted to hospital because of premature thelarche. Serum hormone studies revealed no abnormalities. These findings are similar to those of other authors [5,12,13]. The PR was obviously detected by immunohistochemistry. The same result was reported by Hall et al. [5], who proposed that such findings might indicate some form of hormonal activity by the tumor or sensitivity to hormones originating elsewhere. Signet-ring-like regions may exist in some ovarian stromal tumors, such as OSST, fibroma, Granulosa cell tumor, and signetring stromal tumor. The most important differential diagnosis of
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Y. He et al. / Pathology – Research and Practice 206 (2010) 338–341
Fig. 2. Immunohistochemical findings in the signet-ring-like round and epithelioid cells. (A) Diffuse strong positive staining for vimentin ( 400). (B) Diffuse positivity for calretinin ( 400). (C) Expression of CD56 in tumor cells ( 400). (D) Weakly focal staining for a-inhibin ( 400). (E) Focal staining for PR ( 400). (F) The MIB-1 proliferation index (about 10%) in the tumor cells ( 400).
Table 1 Clinicopathologic features of OSST in adolescent girls [2–4]. Case 1
Case 2
Case 3
Patient’s age (years) Symptom Position Tumor size (cm)
10 Pain in the left lower quadrant and transient suprapubic fullness Left ovary 13 10 8
11 Left gorin pain Left ovary 6.5 3.7 4
Cystic-like change? Immunohistochemistry
Yes None
Yes None
11 Palpated lower abdominal mass Bilateral ovary 17 12 8(right) 8.5 6 5(left) Yes SMA(+)
such signet-ring-like stromal tumors is metastatic mucin-secreting adenocarcinoma (Krukenberg tumor). The negativity of the signet- ring cells for PAS, mucin, EMA, and cytokeratins is strong evidence against Krukenberg tumor. Young and Scully [14] reported that the lutein cells in OSST evolve into a signet-ring-like structure rich in lipids. An electron microscopic study carried out by Lam and Geittmann [11] revealed that OSST consisted of three cell types: lipid-rich cells, fibroblast-like cells, and undifferentiated mesenchymal cells. Su et al. [15] regarded the presence of non-mucin/non-lipid signet-ring cells as the primary characteristic separating signet-ring stromal tumor from the other ovarian sex-cord stromal tumors, and demonstrated that the ultrastructure of the vacuole in the signetring like cells consists of cytoplasmic pseudoinclusions of extracellular edematous matrix. Previously, three possible mechanisms of vacuole formation in signet-ring stromal tumor have been proposed by Dickersin et al. [16], including: general edema of cytoplasmic matrix, dilatation of mitochondria, and invagination of the cell membrane by extracellular matrix. In our case, the signet-ring-like cells inside and outside the cellular nodules showed negative histochemical reactions for lipid, PAS, mucin, and AB stains. These results are in accordance with the features of signet-ring stromal tumor presented in the
literature. However, the pseudolobular growth pattern, cellular nodules, collagenization, and hemangiopericytoma-like vasculature led us to make a final diagnosis of OSST with characteristics of signet-ring stromal tumor. We postulated that, to some extent, OSST and signet-ring stromal tumor assigned to sex-cord stromal neoplasms of the ovary may have overlaps regarding the characteristics of morphology and histochemistry. Our study also shows that these two tumors are not completely independent subtypes and may represent different stages of tumor evolution. More cases and in-depth researches are needed to elucidate the relationship between OSST and signet-ring stromal tumor.
Conflict of interest statement We declare that we have no conflict of interests. References [1] A. Chalvardjian, R.E. Scully, Sclerosing stromal tumors of the ovary, Cancer 31 (1973) 664–670.
ARTICLE IN PRESS Y. He et al. / Pathology – Research and Practice 206 (2010) 338–341
[2] W. Chang, S.J. Oiseth, R. Orentlicher, G. Agarwal, L.J. Yahr, C.G. Cayten, Bilateral sclerosing stromal tumor of the ovaries in a premenarchal girl, Gynecol. Oncol. 101 (2006) 342–345. [3] M.H. Ergeneli, S. Bulut, Sclerosing stromal tumor of the ovary: a case report, Eur. J. Gynaecol. Oncol. 29 (2008) 659–660. [4] N.R. Fefferman, L.P. Pinkney, R. Rivera, D. Popiolek, P. Hummel-Levine, J. Cosme, Sclerosing stromal tumor of the ovary in a premenarchal female, Pediatr. Radiol. 33 (2003) 56–58. [5] O.R. Hall, J.M. Pascasio, J.J. Morrissette, C. Newton, M.Z. Schwartz, J.P. Chadare vian, Study of an ovarian sclerosing stromal tumor presenting as vaginal bleeding in a 7-month-old, Pediatr. Dev. Pathol. 11 (2008) 300–304. [6] I. Ramzy, Signet-ring stromal tumor of ovary: histochemical, light, and electron microscopic study, Cancer 38 (1976) 166–172. [7] M. Matsumoto, Y. Hayashi, Y. Ohtsuki, N. Ikegami, M. Toi, M. Iguchi, M. Hiroi, Signet-ring stromal tumor of the ovary: an immunohistochemical and ultrastructural study with a review of the literature, Med. Mol. Morphol. 41 (2008) 165–170. ˜ o-Enr´ıquez, M. Mart´ınez-Garc´ıa, Signet[8] D. Hardisson, R.M. Regojo, A. Marin ring stromal tumor of the ovary: report of a case and review of the literature, Pathol. Oncol. Res. 14 (2008) 333–336.
341
[9] J.A. Shaw, D.J. Dabbs, K.R. Geisinger, Sclerosing stromal tumor of the ovary: an ultrastructural and immunohistochemical analysis with histogenetic considerations, Ultrastruct. Pathol. 16 (1992) 363–377. [10] L.A. Andrade, A.L. Gentilli, G. Polli, Sclerosing Stromal Tumor in an Accessory Ovary, Gynecol. Oncol. 81 (2001) 318–319. [11] R.M.Y. Lam, P. Geittmann, Sclerosing stromal tumor of the ovary: a light, electron microscopic and enzyme histochemical study, Int. J. Gynecol. Pathol. 7 (1988) 280–290. [12] A. Saitoh, Y. Tsutsumi, R.Y. Osamura, K. Watanabe, Sclerosing stromal tumor of the ovary: immunohistochemical and electron-microscopic demonstration of smooth-muscle differentiation, Arch. Pathol. Lab. Med. 113 (1989) 372–376. [13] G. Marelli, S. Carinelli, A. Mariani, L. Frigerio, A. Ferrari, Sclerosing stromal tumor of the ovary: report of eight cases and review of the literature, Eur. J. Obstet. Gynecol. Reprod. Biol. 76 (1998) 85–89. [14] R.H. Young, R.E. Scully, Differential diagnosis of ovarian tumors based primarily on their pattern and cell types, Semin. Diagn. Pathol. 18 (2001) 161–235. [15] R.M. Su, K.C. Chang, C.Y. Chou, Signet-ring stromal tumor of the ovary: a case report, Int. J. Gynecol. Cancer 13 (2003) 90–93. [16] G.R. Dickersin, R.H. Young, R.E. Scully, Signet-ring stromal and related tumors of the ovary, Ultrasruct. Pathol. 19 (1995) 401–419.
Contents lists available at ScienceDirect
Pathology – Research and Practice journal homepage: www.elsevier.de/prp
Teaching case
Sclerosing stromal tumor of the ovary in a 4-year-old girl with characteristics of an ovarian signet-ring stromal tumor Ying He a, Kai-xuan Yang a,, Wei Jiang a, Dan-qing Wang b, Lei Li a a b
Department of Pathology, West China Second Hospital of Sichuan University, Chengdu, People’s Republic of China Departments of Obstetrics and Gynecology, West China Second Hospital of Sichuan University, The People of South Road 20, Chengdu 610041, People’s Republic of China
a r t i c l e in f o
a b s t r a c t
Article history: Received 18 March 2009 Received in revised form 27 April 2009 Accepted 11 June 2009
Ovarian sclerosing stromal tumor (OSST) is an extremely rare neoplasm that primarily affects young women. Signet-ring stromal tumor is another rare non-functioning benign ovarian stromal neoplasm. We report a case of a right OSST with prominent characteristics of signet-ring stromal tumor in a 4-yearold girl with symptoms of premature thelarche. We describe the clinical, histopathological, and immunohistochemical findings and review the literature. To our knowledge, the 4-year-old patient presented here is the youngest case of OSST reported in premenarchal children. The presence of nonmucin/non-lipid obvious signet-ring-like cells in this case suggests a possible relationship between OSST and signet-ring stromal tumor of the ovary. Crown Copyright & 2009 Published by Elsevier GmbH. All rights reserved.
Keywords: Sclerosing stromal tumor Signet-ring stromal tumor Ovary
Introduction Ovarian sclerosing stromal tumor (OSST), a rare subtype of benign ovarian neoplasm derived from the sex-cord stroma, was first identified by Chalvardjian and Scully in 1973 [1]. It tends to occur in the second to third decade of life. Only three cases have been reported in adolescent girls, as early as 10 years old [2–4]. The youngest case of OSST ever documented is a 7-month-old infant with symptoms of vaginal bleeding at presentation [5]. We describe the case of an OSST excised from a 4-year-old girl, the youngest child patient reported in the English literature. Signet-ring stromal tumor of the ovary is another extremely rare non-functioning distinctive stromal neoplasm that was first described by Ramzy in 1976 [6], with only 11 cases previously reported in the literature [7]. It occurs in adults, affecting patients between 21 and 83 years of age [8].
Clinical history A 4-year-old premenarchal girl was admitted to our hospital because of premature thelarche as diagnosed by her child health doctor. Her previous medical history and her family history were unremarkable. The results of the laboratory studies were as follows: estradiol levels, 37.2 pg/ml; progesterone levels, 0.25 ng/ ml; testosterone levels, 0.07 ng/ml; human chorionic gonadotropin levels, 0 mIU/ml; follicle-stimulating hormone, o1.0 mIU/ml; Corresponding author. Tel.: +86 028 85503177; fax: +86 028 85501359.
E-mail address: [email protected] (K.-x. Yang).
luteinizing hormone, o1.0 mIU/ml; a-fetoprotein, 3.1 ng/ml; prolactin, 4.8 ng/ml. Ultrasonography and computed tomography of the abdomen and pelvic revealed a right ovarian mass approximately 4 cm in diameter with cystic-like degeneration. Under the pre-operative diagnosis of right ovarian tumor, the patient underwent exploratory laparotomy. A smooth mass with welldefined margins, measuring 4.4 3.6 3.4 cm, was found to arise within the right ovary, and was resected. The patient was free of disease, with a follow-up of 4 months.
Materials and methods Tissue was fixed in 10% buffered formalin and embedded in paraffin. Four-micrometer sections were stained with hematoxylin and eosin, as well as with mucicarmine (mucin), PAS with and without diastase digestion, and Alcian blue (AB). For lipid (SudanIII) staining, sections were obtained from frozen tissue. Immunohistochemistry was performed using the avidin–biotin–peroxidase complex method. The antibodies used included vimentin (Dakopatts, Glostrup, Denmark), smooth muscle actin( SMA) (Dako), CD99 (Dako), calretinin (Dako), CD56 (Dako), a-inhibin (Dako), S-100 (Dako), estrogen receptors (ER) (Immunotech), progesterone receptors (PR) (Immunotech), cytokeratins (Dako), and MIB-1 (Zymed, South San Francisco, USA).
Results Macroscopically, the tumor presented as a well-demarcated lump. Sectioning disclosed that tumor was solid, and contained a
0344-0338/$ - see front matter Crown Copyright & 2009 Published by Elsevier GmbH. All rights reserved. doi:10.1016/j.prp.2009.06.005
ARTICLE IN PRESS Y. He et al. / Pathology – Research and Practice 206 (2010) 338–341
339
Fig. 1. (A) Nodular architecture of tumor (H&E 100). (B) Obvious collagenous areas (H&E 400). (C) Hemangiopericytoma-like vasculature (H&E 200). (D) The signetring-like cells possess eccentric nuclei and clear cytoplasm containing single large vacuoles (H&E 400) (indicated by arrows). (E). Cellular nodules transform into hypocellular and hyalinized structures (H&E 200). (F) Signet-ring-like cells are admixed with spindle-shaped cells outside the nodules (H&E 400) (indicated by arrows).
unilocular cyst measuring 1.5 1.0 1.0 cm. The solid component consisted of white-to-pale yellow whorled tissue. No hemorrhagic or necrotic areas were noted. Microscopically, the tumors showed a pseudolobular growth pattern with cellular nodules (Fig. 1A) separated by spindleshaped, fibroblast-like cells, hypocellular areas, collagenous areas (Fig. 1B), and hemangiopericytomatous vasculature (Fig. 1C). The cellular nodules varied in size, and some cellular nodules were primarily composed of round and epithelioid cells containing eccentric nuclei and abundant vacuolated cytoplasm resembling signet-ring cells (Fig. 1D), which eventually transformed into hypocellular and hyalinized structure (Fig. 1E). Signet-ring cells were also admixed with spindle-shaped stromal cells outside the nodules (Fig. 1F). Mitoses were extremely rare. Immunohistochemically, clear vacuoles of the signet-ring-like cells were negative for PAS with and without diastase digestion, lipids, mucin, and AB stains. The signet-ring cells showed diffusely positive immunoreactivity for vimentin (Fig. 2A), SMA, CD99, calretinin (Fig. 2B), CD56 (Fig. 2C), focal staining for a-inhibin (Fig. 2D), S-100 proteins, and PR (Fig. 2E). They were negative for ER and cytokeratins. The fibroblast-like cells only had positivity for Vimentin and were weakly patchy-positive for calretinin. MIB-1 (Ki 67) immunohistochemistry showed a positive reaction in about 10% of the nuclei of the round cells (Fig. 2F).
Discussion Ovarian sclerosing stromal tumor is a rare benign ovarian neoplasm derived from a population of muscle-specific, actinpositive elements from the perifollicular myoid stromal cell [9]. It is usually unilateral, although bilateral cases were reported by Chang et al. [2]. A particularly interesting case of SST arising in an accessory ovary has also been described [10]. Only three cases of OSST have been reported in adolescent girls. The main clinico-
pathologic features of those reports are summarized in Table 1. In our case, positivity for CD99, calretinin, CD56, and a-inhibin in the round tumor cells demonstrated an ovarian stromal origin of the neoplasm. This tumor was characterized by a pseudolobular growth pattern, cellular nodules separated by spindle-shaped stromal cells, varying degrees of collagenization, and hemangiopericytoma-like vasculature. The architecture of cellular nodules found in this case, gradually undergoing loss of cells, leading to hypocellular and hyalinized nodules, was stressed only by Hall et al. [5] and not emphasized in other reports. In the original description, the authors did not mention any hormonal activity of the tumor. However, evidence of hormonal activity has been reported in subsequent cases, showing increased levels of either estrogen, progesterone, or androgens as well as clinical symptoms, including menstrual cycle disturbances, hirsutism, masculinization, precocious puberty, and vaginal bleeding [5]. The cells of OSST were enzymatically active for estradiol 17-bdehydrogenase, glucose-6-phosphate dehydrogenase, and isoligandin citric dehydrogenase [11]. This result showed that the tumor cells can synthesize dehydroepiandrosterone and estradiol from circulating estrone. The signet-ring stromal tumors of the ovary reported so far were hormonally inactive, although the progesterone receptors were found to be strongly and uniformly positively expressed [7]. In this case, the girl was admitted to hospital because of premature thelarche. Serum hormone studies revealed no abnormalities. These findings are similar to those of other authors [5,12,13]. The PR was obviously detected by immunohistochemistry. The same result was reported by Hall et al. [5], who proposed that such findings might indicate some form of hormonal activity by the tumor or sensitivity to hormones originating elsewhere. Signet-ring-like regions may exist in some ovarian stromal tumors, such as OSST, fibroma, Granulosa cell tumor, and signetring stromal tumor. The most important differential diagnosis of
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Y. He et al. / Pathology – Research and Practice 206 (2010) 338–341
Fig. 2. Immunohistochemical findings in the signet-ring-like round and epithelioid cells. (A) Diffuse strong positive staining for vimentin ( 400). (B) Diffuse positivity for calretinin ( 400). (C) Expression of CD56 in tumor cells ( 400). (D) Weakly focal staining for a-inhibin ( 400). (E) Focal staining for PR ( 400). (F) The MIB-1 proliferation index (about 10%) in the tumor cells ( 400).
Table 1 Clinicopathologic features of OSST in adolescent girls [2–4]. Case 1
Case 2
Case 3
Patient’s age (years) Symptom Position Tumor size (cm)
10 Pain in the left lower quadrant and transient suprapubic fullness Left ovary 13 10 8
11 Left gorin pain Left ovary 6.5 3.7 4
Cystic-like change? Immunohistochemistry
Yes None
Yes None
11 Palpated lower abdominal mass Bilateral ovary 17 12 8(right) 8.5 6 5(left) Yes SMA(+)
such signet-ring-like stromal tumors is metastatic mucin-secreting adenocarcinoma (Krukenberg tumor). The negativity of the signet- ring cells for PAS, mucin, EMA, and cytokeratins is strong evidence against Krukenberg tumor. Young and Scully [14] reported that the lutein cells in OSST evolve into a signet-ring-like structure rich in lipids. An electron microscopic study carried out by Lam and Geittmann [11] revealed that OSST consisted of three cell types: lipid-rich cells, fibroblast-like cells, and undifferentiated mesenchymal cells. Su et al. [15] regarded the presence of non-mucin/non-lipid signet-ring cells as the primary characteristic separating signet-ring stromal tumor from the other ovarian sex-cord stromal tumors, and demonstrated that the ultrastructure of the vacuole in the signetring like cells consists of cytoplasmic pseudoinclusions of extracellular edematous matrix. Previously, three possible mechanisms of vacuole formation in signet-ring stromal tumor have been proposed by Dickersin et al. [16], including: general edema of cytoplasmic matrix, dilatation of mitochondria, and invagination of the cell membrane by extracellular matrix. In our case, the signet-ring-like cells inside and outside the cellular nodules showed negative histochemical reactions for lipid, PAS, mucin, and AB stains. These results are in accordance with the features of signet-ring stromal tumor presented in the
literature. However, the pseudolobular growth pattern, cellular nodules, collagenization, and hemangiopericytoma-like vasculature led us to make a final diagnosis of OSST with characteristics of signet-ring stromal tumor. We postulated that, to some extent, OSST and signet-ring stromal tumor assigned to sex-cord stromal neoplasms of the ovary may have overlaps regarding the characteristics of morphology and histochemistry. Our study also shows that these two tumors are not completely independent subtypes and may represent different stages of tumor evolution. More cases and in-depth researches are needed to elucidate the relationship between OSST and signet-ring stromal tumor.
Conflict of interest statement We declare that we have no conflict of interests. References [1] A. Chalvardjian, R.E. Scully, Sclerosing stromal tumors of the ovary, Cancer 31 (1973) 664–670.
ARTICLE IN PRESS Y. He et al. / Pathology – Research and Practice 206 (2010) 338–341
[2] W. Chang, S.J. Oiseth, R. Orentlicher, G. Agarwal, L.J. Yahr, C.G. Cayten, Bilateral sclerosing stromal tumor of the ovaries in a premenarchal girl, Gynecol. Oncol. 101 (2006) 342–345. [3] M.H. Ergeneli, S. Bulut, Sclerosing stromal tumor of the ovary: a case report, Eur. J. Gynaecol. Oncol. 29 (2008) 659–660. [4] N.R. Fefferman, L.P. Pinkney, R. Rivera, D. Popiolek, P. Hummel-Levine, J. Cosme, Sclerosing stromal tumor of the ovary in a premenarchal female, Pediatr. Radiol. 33 (2003) 56–58. [5] O.R. Hall, J.M. Pascasio, J.J. Morrissette, C. Newton, M.Z. Schwartz, J.P. Chadare vian, Study of an ovarian sclerosing stromal tumor presenting as vaginal bleeding in a 7-month-old, Pediatr. Dev. Pathol. 11 (2008) 300–304. [6] I. Ramzy, Signet-ring stromal tumor of ovary: histochemical, light, and electron microscopic study, Cancer 38 (1976) 166–172. [7] M. Matsumoto, Y. Hayashi, Y. Ohtsuki, N. Ikegami, M. Toi, M. Iguchi, M. Hiroi, Signet-ring stromal tumor of the ovary: an immunohistochemical and ultrastructural study with a review of the literature, Med. Mol. Morphol. 41 (2008) 165–170. ˜ o-Enr´ıquez, M. Mart´ınez-Garc´ıa, Signet[8] D. Hardisson, R.M. Regojo, A. Marin ring stromal tumor of the ovary: report of a case and review of the literature, Pathol. Oncol. Res. 14 (2008) 333–336.
341
[9] J.A. Shaw, D.J. Dabbs, K.R. Geisinger, Sclerosing stromal tumor of the ovary: an ultrastructural and immunohistochemical analysis with histogenetic considerations, Ultrastruct. Pathol. 16 (1992) 363–377. [10] L.A. Andrade, A.L. Gentilli, G. Polli, Sclerosing Stromal Tumor in an Accessory Ovary, Gynecol. Oncol. 81 (2001) 318–319. [11] R.M.Y. Lam, P. Geittmann, Sclerosing stromal tumor of the ovary: a light, electron microscopic and enzyme histochemical study, Int. J. Gynecol. Pathol. 7 (1988) 280–290. [12] A. Saitoh, Y. Tsutsumi, R.Y. Osamura, K. Watanabe, Sclerosing stromal tumor of the ovary: immunohistochemical and electron-microscopic demonstration of smooth-muscle differentiation, Arch. Pathol. Lab. Med. 113 (1989) 372–376. [13] G. Marelli, S. Carinelli, A. Mariani, L. Frigerio, A. Ferrari, Sclerosing stromal tumor of the ovary: report of eight cases and review of the literature, Eur. J. Obstet. Gynecol. Reprod. Biol. 76 (1998) 85–89. [14] R.H. Young, R.E. Scully, Differential diagnosis of ovarian tumors based primarily on their pattern and cell types, Semin. Diagn. Pathol. 18 (2001) 161–235. [15] R.M. Su, K.C. Chang, C.Y. Chou, Signet-ring stromal tumor of the ovary: a case report, Int. J. Gynecol. Cancer 13 (2003) 90–93. [16] G.R. Dickersin, R.H. Young, R.E. Scully, Signet-ring stromal and related tumors of the ovary, Ultrasruct. Pathol. 19 (1995) 401–419.