Document Type : Case Report Article
Authors
1 Department of Obstetrics and Gynecology, Isfahan University of Medical Sciences, Isfahan, Iran
2 Departments of Pathology, Isfahan University o Medical Sciences, Isfahan, Iran
Abstract
Signet ring stromal tumor is a rare benign ovarian neoplasm, of which only about 17 cases have been reported since 1996. The signet ring appearance of this tumor may mimic a Krukenberg tumor and result in a diagnostic challenge in some cases. The previous cases occurred in adult or in old patients. We have reporteda Signet ring stromal tumor in a 13-year-old girl.
Highlights
✅ Signet ring stromal tumor is a rare benign ovarian neoplasm, of which only about 17 cases have been reported since 1996.
Keywords
Main Subjects
Signet ring stromal tumor is a rare benign ovarian neoplasm which was reported by Ramzy in 1996 for the first time (1). Only about 17 cases have been reported since 1996, listed in Table 1 (1-14). The signet ring appearance of this tumor may mimic a Krukenberg tumor and result in a diagnostic challenge in some cases, specially if the neoplasm involves both ovaries (13).
Table 1. Clinical features of signet ring stromal tumor from review of literature
| Author | Presentation | Laterality | Macroscopy | Size | Procedure | Outcome |
| Ramzy, 1976 (1) | 28Yrs, Abdominal pain |
Right-sided | Solid | 8 cm | Total abdominal hysterectomy and right salpingo-oophorectomy, wedge resection of left ovary | No recurrence until 15 months after surgery |
| Suarez et al., 1992 (2) |
50Yrs, Menopause3 years ago , Abdominal pain, Palpable mass at left hemiabdomen |
Solid white, with irregular yellowish areas, firm fasciculated in appearance |
9 cm | Simple left oophorectomy | No recurrence until 26 months after surgery | |
| Dickersin et al.,1995 (3) | 1.21Yrs,NA 2.78Yrs,NA 3.83Yrs,NA |
Solid Hemorrhagic Solid cystic Solid |
5 cm 8.5 cm 8.9 cm |
NA NA NA |
No recurrence for 2 years after surgery No recurrence for 1.6 years after surgery No recurrence for 2 months after surgery |
|
| Cashel et al., 1999 (4) |
52Yrs, weight loss, Left sided adnexal mass, No hormonal effect |
Conjunction with Brenner tumor | A hard well-defined nodule with a firm mottled yellow to tan surface | 2.5 cm | Total abdominal hysterectomy and bilateral salpingo-oophorectomy | No evidence of recurrence in 2 years follow up |
| Su et al., 2003 (5) |
76Yrs, Low abdominal pain, Left palpable adnexal mass |
Left-sided | Grayto pale-tan solid tumor with Focal stromal edema and scattered chalky-white spots |
5 cm | Bilateral salpingo-oophorectomy | No recurrence for 12 months after surgery |
| Vang et al., 2004 (6) |
1.34 Yrs,pelvic mass 2.35 Yrs,pelvic mass 3.41 Yrs,Abdominal pain |
Left-sided Right sided Right sided |
Gray, tan, or yellow. One tumor had a nodular appearance and two were focally hemorrhagic and necrotic. |
13 cm 3.5 cm 13 cm |
1 and 3:Total abdominal hysterectomy and bilateral salpingo-oophorectomy 2:Right oophorectomy |
No recurrence for 17.4 years after surgery No recurrence for 4.7 years after surgery No recurrence for 1month after surgery |
| Hardisson et al., 2008 (7) | 54Yrs, G2P2 3month history of low abdominal discomfort, Palpable mass at left hemiabdomen |
Left sided | Solid | 5 cm | Laparoscopic bilateral salpingo-oophorectomy | No recurrence for 21 months after surgery |
| Matsumoto et al., 2008 (8) |
76Yrs, Incidental pelvic mass in examination |
Left-sided | Solid light brown focally admixed with white fibromatous tissue |
7 cm | Total abdominal hysterectomy and bilateral salpingo-oophorectomy | No recurrence for 9 months after surgery |
| Forde et al., 2010 (9) |
69 y, G5 Hx of hysterectomy Abdominal pain of 1 week duration, No findings in Examination secondry to body habitus |
Bilateral | 5 cm | Exploratory laparatomy, partial omentectomy, bilateral salpingo-oophorectomy and peritoneal washing | - | |
| Sukur et al., 2010 (10) |
44Yrs, G4P2 Polymenorrheafor 3 months, Palpable right adnexal mass |
Right-sided | Semisolid | 5 cm | Right salpingo-oophorectomy | - |
| Kopszynski et al., 2016 (12) |
79Yrs Abdominal uterine bleeding, Normal pelvic examination |
Left sided | A poorly circumscribed, yellowish, firm, solid tumor | 1.1 cm | Abdominal hysterectomy and bilateral salpingo-oophorectomy | No recurrence after 11 months |
| McGregor et al., 2016 (11) |
64 Yrs, left ovarian mass identified by imaging |
Left sided | Yellow-tan and gray-white areas | 3.5 cm | Total hysterectomy and bilateral salpingo-oophorectomy | - |
| 2020 (13) | 70 Yrs, Abdominal pain, distention, bloating and rectal bleeding |
Bilateral tumor | Solid homogenous white-yellow, firm cut surfaces | Left:4 Right 5.5 cm |
Total abdominal hysterectomy and bilateral salpingo-oophorectomy | No recurrence for 12 months after surgery |
| Tsai et al., 2020 (14) |
43y Palpable pelvic mass |
Left-sided Collision tumor of sclerosing stromal tumor and signet-ring stromal tumor |
Cystic and solid | 16 cm | Left salpingo-oophorectomy | - |
Signet ring stromal tumor is a rare ovarian stromal neoplasm (7) Up to now only 17 cases have been reported (1-14). All the reported cases have occurred in adults with a range between 21 and 83 years of age (Mean age of 54 years) (7). The clinical features are nonspecific,and abdominal pain is the most common symptom. On imaging,the mass may be solid or cystic ,nevertheless there is no specific features for distinguishing this tumor from other neoplasms. Grossly, the tumor size varies from 1.1 to 16 cm (12,14). Although bilaterally have been reported in two cases (9,13), most of them are unilateral. This tumor mostly shows a solid cut surface, cystic and hemorrhagic changes also are noted (3,6,13). All patients have no evidence of recurrence or metastasis after surgery (Duration of follow-up ranges from 1 month to 17.4 years). Cashel et al., reported a signet cell stromal tumor in association with Brenner tumor in 1999 (4). Tsai et al reported a collision tumor of Sclerosing stromal tumor and Signet ring stromal tumor in 2019 (14). Microscopically tumor is composed of different proportion of spindle and round cells which some of them have similar morphology of signet ring cells. Marked cellular atypia, mitosis, and necrosis are absent. In some cases PAS positive intracytoplasmic hyaline globules are noted (2). Special staining for mucin and lipid are negative. Ultrastructural studies have shown, the vacuoles are derived from diffuse cytoplasmic edema, mitochondrial swelling or cytoplasmic pseudoinclusions of extracellular matrix (2,3,4,5). Histochemical and immunohistochemical findings have been shown in Table 2. In all studies tumoral cells are negative for EMA, CK, and CEA. Immunoreactivity for calretinin, inhibin, ER, cyclin D1 and beta-catenin has contradictory. The most important differential diagnosis of signet ring stromal tumor is Krukenberg tumor. Most of the patients with Krukenberg tumor have no previous history of carcinoma. Though bilaterality has been reported in both tumors, therefore it is not useful for differentiation of these two tumors. Grossly the gelatinous appearance favors krukenberg tumor but, multinodularity, necrosis, and hemorrhage may be seen in both neoplasms. Krukenberg tumor are constantly positive for PAS-D and mucicarmin. On the other hand,all signet ring stromal tumors are negative. Pancytokeratin and Vimentin are so helpful for distinguishing of challenging cases, so that keratin is not expressed by signet ring stromal tumors whereas Vimentin is strongly expressed (6).
Signet ring stromal tumor is a rare stromal neoplasm. Previous reported cases aged more than 21 years old. we report a rare case in a 13-year-oldgirl. This benign neoplasm may be misdiagnosed as krukenberg tumor with aggressive behavior, but Histochemical and immunohistochemical techniques are helpful for differentiation between these tumors.
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The authors declare no conflicts of interest.
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