Signet ring cell carcinoma of the testis: clinicopathologic and molecular evidence for germ cell tumor origin--a case report

Development of a somatic-type malignancy from a mixed germ cell tumor is a rare but recognized event and typically represented by sarcoma or, less commonly, by carcinoma. This phenomenon is generally believed to result from progression of a teratomatous component. In many cases, because of intermingling of other germ cell tumor components, the diagnosis is apparent; however, in rare cases, metastatic carcinoma to the testis or a novel primary tumor may be a diagnostic consideration. In this study, we report the clinicopathologic, immunohistochemical, and molecular features of a 53-year-old man, whose testicular tumor was composed entirely of signet ring cells, mimicking metastatic carcinoma. Subsequent retroperitoneal lymph node dissection revealed metastatic deposits composed of teratoma and yolk sac tumor, in addition to signet ring cell carcinoma. Fluorescence in situ hybridization for abnormalities of chromosome 12p revealed the presence of i(12p) in both the teratoma and signet ring cell carcinoma in the metastasis and in signet ring cells in the testis, supporting a common germ cell origin. Our report indicates that signet ring carcinoma cells in an orchiectomy specimen, although usually strongly suggestive of metastatic adenocarcinoma from a primary tumor in another organ, may be a primary testicular neoplasm of germ cell tumor origin. This is the first report of testicular signet ring cell carcinoma of germ cell tumor derivation.     

Stage pT1 signet ring cell carcinoma of the urinary bladder: a case report

A case of primary signet ring cell carcinoma of the urinary bladder is described. A 59-year-old man presented with microscopic hematuria, and cystoscopy revealed a white nonpapillary tumor. Histopathological examination of the resected tumor revealed signet ring cell carcinoma and transitional cell carcinoma. Histological depth of invasion was pT1. No adjuvant therapy was performed. Primary signet ring cell of the urinary bladder is a rare tumor with 37 cases reported to date in Japan. We investigated previously reported cases and discussed adjuvant therapies of superficial signet ring cell carcinoma of the urinary bladder.     

Primary carcinoid tumor arising in a retroperitoneal mature teratoma in an adult

An extremely rare case of a primary carcinoid tumor arising in a mature retroperitoneal teratoma is reported. A 53-year-old woman was admitted for further examination of an incidental retroperitoneal mass with calcification. Computed tomography scans demonstrated a tumor with fat, soft tissue and bone densities on the left renal hilum. Surgical excision of the tumor was performed with a preoperative diagnosis of retroperitoneal teratoma. The pathological diagnosis was mature teratoma, including all three germ layers. A carcinoid tumor was evident among teratoid tissues and it was thought to be a teratoma with malignant transformation. The patient did not have a carcinoid syndrome and had an uneventful recovery. She has been followed for 31 months with no recurrence. Carcinoid tumors rarely occur in teratomas of the ovary and the testis and, to our knowledge, this is the first case of carcinoid arising in a retroperitoneal mature teratoma.     

Signet ring cell carcinoma of bladder.

The ninth reported case of primary signet ring cell carcinoma of the bladder is described. This particular tumor, whose histology was examined repeatedly during its evolution, showed an interesting change in microscopic appearance after irradiation. The emerging features of signet ring cell carcinoma of the bladder are discussed.     

Superficial and pedunculated signet ring cell carcinoma of the urinary bladder: a case report]

A case of primary signet ring cell carcinoma of the urinary bladder is described. A 63-year-old man presenting with difficulty of urination and miction pain had a pedunculated soybean-size tumor on the left lateral wall of the bladder. Specimens of the tumor were obtained by transurethral resection and the pathological diagnosis was signet ring cell carcinoma. There was no evidence of bladder metastasis from other organs. The patient then had intraoperative radiotherapy and he is alive without recurrence 20 months after the operation. We briefly discuss 73 cases of signet ring cell carcinoma of the urinary bladder collected from the English and Japanese literature. The tumor in this patient was the smallest of all cases reported previously.     

Primary carcinoid tumor in a retroperitoneal mature teratoma: report of a case

Primary retroperitoneal teratoma in an adult is rare, as is the occurrence of a malignant tumor within a mature teratoma, known as “malignant transformation”. A 24-year-old woman was admitted to our hospital for investigation of an abdominal mass. Computed tomography and magnetic resonance imaging revealed a multilocular mass in the right upper abdomen. The tumor consisted of fat, soft tissue, and bone, with a slightly enhanced solid component. The tumor was diagnosed preoperatively as a retroperitoneal mature teratoma with an immature component, and excised. Histologically, it was composed mainly of mature fat, soft tissue, and bone, accompanied by a solid component of prostate-like tissue. In addition, a latent carcinoid tumor was recognized in the middle of the tumor. The tumor was finally diagnosed as a primary carcinoid tumor within the retroperitoneal mature teratoma. The patient has been followed-up for 24 months since her operation without any evidence of recurrence. We report this case to highlight the possibility of malignant transformation in adult retroperitoneal teratoma, even when the preoperative diagnosis is benign mature teratoma.     

Signet ring cell carcinoma in adenomatous polyp at site of ureterosigmoidostomy 16 years after conversion to ileal conduit

The case of a signet ring cell carcinoma of the colon arising in an adenoma at the site of ureterosigmoidostomy after conversion to an ileal conduit is reported. Urine exposure to the colonic mucosa was present only for a short period before development of an adenoma with subsequent signet ring cell carcinoma transformation more than 15 years later. In the face of recent reports on adenocarcinoma in cases of bladder substitution or augmentation without any possible promoting influence of the fecal stream, the well known risk of neoplasia after sigmoidostomy remains unclear in its etiology.     

Primary signet ring cell carcinoma of bladder exemplifying vesical epithelial multipotentiality.

The eighth reported case of primary signet ring cell carcinoma of the urinary bladder is described clinically and pathologically. It is unusual because of complete replacement of the transitional epithelium by squamous and glandular metaplasia. The latter was particularly striking, demonstrating "normal" colonic mucosa, cystitis glandularis, well-differentiated adenocarcinoma, poorly differentiated adenocarcinoma, and the rare signet ring cell carcinoma variant. The possible origin of such changes is discussed.     

Extraordinary large primary retroperitoneal cystic teratoma: An extremely rare neoplasm

Teratomas are the germ cell tumors which comprises of tissues from all the three germ cell layers. Primary retroperitoneal teratoma is a relatively rare tumor in adults. The primary retroperitoneal teratoma constitutes 6–11% of the retroperitoneal tumors. Sixty percent of the retroperitoneal teratomas occur in children less than 15 years.It occurs more commonly in females than males. The order of frequency for teratoma is ovary, testis, mediastinum and the retroperitoneum at last. Early diagnosis and surgery are the mainstay of treatment. We report a rare case of large primary retroperitoneal teratoma. This is the largest primary retroperitoneal teratoma reported in the literature to our knowledge.     

Primary signet ring cell adenocarcinoma of the bladder

Primary signet ring cell adenocarcinoma of the bladder accounts for less than 1 per cent of all primary bladder neoplasms. This tumor is insidious because of its subepithelial infiltrative nature, which makes diagnosis possible only late in the course of the disease. Survival is poor; greater than 50 per cent of the patients are dead within a year after diagnosis. Exenterative procedures offer the only hope of palliation; irradiation and chemotherapy have not been effective. We add 5 cases of primary signet ring cell adenocarcinoma of the bladder and 1 case of high grade transitional cell carcinoma of the bladder with signet ring cell foci to the 14 cases reported in the literature. Pathological correlation supports the origin of this neoplasm from totipotential transitional epithelium.     

A case of primary signet ring cell carcinoma of the urinary bladder presenting as nocturnal incontinence

This is a report of our experience of a case of primary signet ring cell carcinoma of the urinary bladder. The patient was a 56-year-old man who was referred to our hospital presenting with incontinence and lumbago. A drip infusion pyelography study indicated bilateral hydronephrosis and a contracted bladder. Signet ring cell carcinoma was observed in the bladder submucosa after the second transurethral biopsy set. No other cancer lesions could be identified even after careful examination. Therefore, it was diagnosed as a primary signet ring cell carcinoma of the bladder. Total cystectomy with bilateral uretero-cutaneostomy reconstruction was performed. The pathological stage was T4bN0M0 and the surgical margin was positive at the site of the pubic bone. Adjuvant therapy was not given. There was no evidence of disease 20 months after the operation. In Japan, this is the third case of primary signet ring cell carcinoma of the bladder presenting as incontinence.     

A case of signet ring cell carcinoma of the urinary bladder

A 60-year-old female complained of gross hematuria and urinary frequency on November 27th, 1982. Cystoscopic examination revealed papillary invasive tumor around the bladder neck and a transurethral biopsy showed signet ring cell carcinoma. Since there was no adenocarcinoma in any other organs, we diagnosed it as primary signet ring cell carcinoma of the urinary bladder. Total cystectomy with ileal conduit and post-operative irradiation were performed, but she died on May 13th, 1983. We summarize 16 cases of primary signet ring cell carcinoma of the urinary bladder including this case and discuss this rare condition.     

Signet ring cell carcinoma of the breast as a source of pelvic floor metastatic mass. A case report

Primary signet ring cell carcinoma of the breast is a very rare tumour. We present a case with pure signet ring cell carcinoma of the breast, which was recognized as metastasis on the pelvic floor, before developing breast symptoms and signs. A 40-year old woman was admitted with abdominal pain. First diagnostic effort revealed a cystic mass on the pelvic floor, compressing the colon and other neighbouring organs. A biopsy of the pelvic mass was performed. The histopathological examination revealed metastatic signet-ring cell carcinoma. At the time of the first operation, the mammary glands were not suspicious. No other sources of primary tumour were evidenced. An inflammatory sign developed in right breast two months after biopsy of the pelvic metastasis. The histopathology of the breast incisional biopsy revealed primary pure signet ring cell carcinoma of the breast. Because the oestrogen and progesterone receptor were negative in the tumoral tissue, the patient underwent chemotherapy followed by modified radical mastectomy, chemotherapy, and palliative resection of the metastatic mass. The patient was followed up for eight months. To our knowledge, in English literature, we believe that this case is the first report of signet ring cell carcinoma of the breast presenting with pelvic floor metastasis without breast sign.     

Primary signet ring cell carcinoma of the bladder: a case report

A 71-year-old man complained of dyspnea and general fatigue. His blood tests showed severe renal dysfunction. Computed tomographic scan, bone scintigram, and cystoscopy revealed primary signet ring cell carcinoma of the urinary bladder with multiple bone metastases (cT2N0M1). As the general condition of the patient was poor, nephrostomy was performed. He died one month after the diagnosis due to cancer progression. The prognosis of signet ring cell carcinoma of the bladder is poor because many cases presented at an advanced stage. Fifty cases of signet ring cell carcinoma in the urinary bladder reported in Japan are reviewed.     

原发性前列腺印戒细胞癌(附二例报告及文献复习)

目的：探讨原发性前列腺印戒细胞癌的临床特点。方法：分析2例原发性前列腺印戒细胞癌患者的临床资料，结合文献得以讨论。结果：2例患者年龄分别为64岁和73岁，临床表现为排尿困难、膀胱刺激症及会阴不适。组织学：印戒状癌细胞呈圆形，胞质丰富透明，胞核呈新月状位于细胞一侧，核分裂像多见。免疫组化染色PCA（ ）、PAP( )、AR( )、低分子CK( )，CEA（-）、AB/PAS（-）。1例D期患者行双侧睾丸切除及氟他胺治疗，术后6个月死于广泛转移；1例B2期患者行根治性前列腺切除、双侧睾丸切除、内分泌和局部放射治疗，随访25个月未见复发和转移。结论：原发性前列腺印戒细胞癌是一种罕见、高度恶性的肿瘤，确诊需依赖组织病理学及免疫组织化学检查。     

Primary signet ring cell carcinoma of the prostate: Report and review of 42 cases

We report a case of primary signet ring cell carcinoma of the prostate in a 75-year-old man. Serum prostate specific antigen (PSA) level at presentation was 9.3 ng/mL. The tumor was confined within the right prostate lobe and the patient was treated with neoadjuvant hormonal therapy and radical prostatectomy. He was alive with no evidence of disease 12 months after surgery. None of the tumor was stained with periodic acid-Schiff and Alcian blue. Immunohistochemically, the tumor was positive for PSA and prostatic acid phosphatase and negative for carcinoembryonic antigen. We reviewed 41 previously reported cases of signet ring cell carcinoma of the prostate, examining both histopathological and clinical information.     

Primary ovarian mucinous tumors with signet ring cells: report of 3 cases with discussion of so-called primary Krukenberg tumor

The distinction between a primary ovarian mucinous carcinoma or even a borderline mucinous tumor and a metastatic mucinous carcinoma may be difficult. A constellation of clinical, gross pathologic and morphologic features is used in this distinction. One of the most important morphologic features suggesting a metastatic mucinous carcinoma in the ovary is the presence of signet ring cells; these are considered rare in primary ovarian mucinous tumors. In this study, we report 3 primary ovarian mucinous tumors with a component of signet ring cells. The tumors arose in patients aged 27, 55, and 60, were unilateral, confined to the ovary and stage IA. They ranged from 9 to 27 cm; 1 was grossly a multiloculated cystic lesion and 2 were cystic and solid. In one case, the neoplasm had the architecture of a mucinous adenofibroma but had frankly malignant cells lining glands and forming solid aggregates of cells. A second tumor also had the background of an adenofibroma. The third was mostly a mucinous cystadenoma. In one case, endometriosis was present in the same ovary; teratomatous elements were not identified in any case. Immunohistochemistry, performed in 2 cases, showed both to be diffusely positive with CK7 and CA19.9, including the signet ring cells. CK20 was positive in both cases (1 focal; 1 diffuse). Estrogen receptor and CA125 were diffusely positive and carcinoembryonic antigen and CDX2 focally positive in 1 case. Chromogranin and synaptophysin were negative. Investigations to exclude a gastrointestinal neoplasm in 2 cases were negative. Features favoring a primary rather than a metastatic neoplasm are unilateral tumor, low stage, background of adenofibroma or cystadenoma, associated endometriosis in 1 case and an absence of features which are characteristic of secondary mucinous carcinomas in the ovary, such as surface tumor deposits, a nodular growth pattern, and lymphovascular permeation. Immunohistochemistry is of limited value because of overlapping immunophenotype between a primary ovarian mucinous tumor and a metastasis from the stomach, pancreas, biliary tree, appendix, or colorectum, the most likely primary sites for a secondary exhibiting similar features. Our study illustrates that signet ring cells occur rarely in a primary ovarian mucinous tumor; even when conspicuous the features differ from those of the usual Krukenberg tumor. At least some cases of so-called primary Krukenberg tumor may be similar to our cases. However, the designation primary Krukenberg tumor should not be used as, apart from the signet ring cells, a resemblance to a "true" Krukenberg tumor of the secondary type is limited. The tumors should be classified according to the underlying background neoplasm with a notation concerning the signet ring cell component.     

Retroperitoneal mixed germ cell tumor mimicking a renal neoplasm: a case report

Retroperitoneal malignant germ cell tumors (GCTs) are rare and of uncertain origin. Almost all retroperitoneal GCTs with seminomatous histology have been reported in male patients. Pediatric hypertension in a girl secondary to a retroperitoneal mixed GCT (dysgerminoma plus mature monodermal teratoma) has not been reported previously in the literature. We present the first case of an 11-year-old girl with a retroperitoneal mixed GCT presenting with hypertension.     

Signet ring cell carcinoma of a pulled-through sigmoid colon mimicking a primary invasive bladder tumor: case report and review of the literature

Primary signet ring cell carcinoma of the urinary bladder and colon are rare disease entities that are aggressive, difficult to manage, and portend a poor prognosis. We present a case report of a 25-year-old man born with an imperforate anus who developed signet ring cell carcinoma of the pulled-through sigmoid colon that mimicked a primary invasive bladder tumor. Despite radical surgery and adjuvant radiation, the patient died of his disease 7 months after surgery.