Prognostic factors in patients with uterine sarcoma: the SARCUT study.

OBJECTIVE: Uterine sarcomas are a rare and heterogeneous group of malignancies that include different histological sub-types. The aim of this study was to identify and evaluate the impact of the different prognostic factors on overall survival and disease-free survival of patients with uterine sarcoma. METHODS: This international multicenter retrospective study included 683 patients diagnosed with uterine sarcoma at 46 different institutions between January 2001 and December 2007. RESULTS: The 5-year overall survival for leiomyosarcoma, endometrial stromal sarcoma, undifferentiated sarcoma, and adenosarcoma was 65.3%, 78.3%, 52.4%, and 89.5%, respectively, and the 5-year disease-free survival was 54.3%, 68.1%, 40.3%, and 85.3%, respectively. The 10-year overall survival for leiomyosarcoma, endometrial stromal sarcoma, undifferentiated sarcoma and adenosarcoma was 52.6%, 64.8%, 52.4%, and 79.5%, respectively, and the 10-year disease-free survival was 44.7%, 53.3%, 40.3%, and 77.5%, respectively. The most significant factor associated with overall survival in all types of sarcoma except for adenosarcoma was the presence of residual disease after primary treatment. In adenosarcoma, disease stage at diagnosis was the most important factor (hazard ratio 17.7; 95% CI 2.86 to 109.93). CONCLUSION: Incomplete cytoreduction, tumor persistence, advanced stage, extra-uterine and tumor margin involvement, and the presence of necrosis were relevant prognostic factors significantly affecting overall survival in uterine sarcoma. The presence of lymph vascular space involvement and administration of adjuvant chemotherapy were significantly associated with a higher risk of relapse.

[Interdisciplinary S2k guidelines on the diagnosis and treatment of uterine sarcomas-recommendations for surgical pathology]. / S2k-Leitlinie Diagnostik und Therapie uteriner Sarkome ­ Anforderungen an die Pathologie.

Uterine sarcomas represent a heterogeneous group of rare malignancies, derived from the myometrium, the endometrial stroma, and very rarely from the nonspecialized uterine soft tissue. The actual incidence is about 1.5 for Caucasian and 3.0 for Afro-American women. There is no grading system for leimoysarcoma defined by the WHO classification; however, if clinicians request, the FNCLCC grading can be specified in analogy to soft tissue sarcomas. Adenosarcomas must be distinguished from adenofibromas (the existence of which is questionable)-with the vast majority of these tumors being uterine adenosarcomas. Within adenosarcomas, deep myometrial invasion (>50%), sarcomatous overgrowth, and a high-grade heterologous component are associated with a higher recurrence rate and poor survival. The immunohistochemical panel represents a very helpful tool for distinguishing low-grade from high grade endometrial stromal sarcomas (ESS) and may be supplemented by molecular analyses. Steroid hormone receptor analysis should be performed for all ESS due to the possible therapeutic relevance. Undifferentiated uterine sarcomas represent a diagnosis of exclusion and have a very poor prognosis. Carcinosarcomas represent a special subtype of endometrial carcinomas and are in fact not uterine sarcomas. Uterine sarcomas may present substantial intratumoral heterogeneity and adequate embedding is mandatory. Lesions ≤2 cm in the largest dimension should be processed completely and larger tumors should be processed with one block per centimeter for the largest tumor dimension.

Conservative management of uterine adenosarcoma: lessons learned from 20 years of follow-up.

PURPOSE: Uterine adenosarcomas (UAs) account for 5-8% of cases of uterine sarcomas. Treatment includes total abdominal hysterectomy (TAH) and bilateral salpingo-oophorectomy (BSO). Fertility preservation is an emerging concept in gynaecology oncology and is particularly relevant in UA, where cases are diagnosed as young as 15-year-old. This manuscript demonstrates a case of UA which was treated conservatively, achieved successful livebirths and underwent completion hysterectomy after two decades of follow-up. METHOD: This was a retrospective case note review. RESULTS: An 18-year-old nulliparous woman presented with abnormal vaginal bleeding. Ultrasound identified an endometrial polyp, which was histologically diagnosed as low-grade adenosarcoma. She was advised to undergo TAH and BSO, but instead decided to preserve her fertility and opted for conservative management. She was monitored with pelvic ultrasound, hysteroscopy and endometrial biopsy bi-annually, with annual pelvic magnetic resonance imaging for 10 years which was uneventful. 11 years post-operatively she conceived following in-vitro fertilization (IVF) but suffered a miscarriage at 16 weeks likely due to cervical incompetence. She subsequently conceived with twins. She delivered spontaneously preterm at 28 weeks. Both children are alive and well. After 20 years of follow-up, she underwent a laparoscopic hysterectomy with no evidence of recurrence. She remains disease free. CONCLUSION: Whilst radical completion surgery should be advised in UA, this case, in addition to all published conservatively managed cases of UA, demonstrates that conservative management is possible in appropriately selected women. Intensive monitoring post-operatively is essential owing to the risk of recurrence; however, this may pose deleterious side effects which require consideration.

The Importance of Lymphovascular Invasion in Uterine Adenosarcomas: Analysis of Clinical, Prognostic, and Treatment Outcomes.

OBJECTIVE: This retrospective study examined the clinicopathologic features of adenosarcoma patients to determine potential prognostic factors and retrospectively evaluated overall survival (OS), disease-free survival (DFS), and local recurrence-free survival (LRFS) after primary treatment of adenosarcoma including surgery, radiation, and chemotherapy. METHODS: One hundred sixty-five patients with adenosarcoma were identified from the MD Anderson Cancer Center tumor registry between 1982 and 2014. Clinical data were collected retrospectively. Pathologic characteristics were examined by sarcoma pathologists. We used the Kaplan-Meier method to estimate OS, DFS, and LRFS. The log-rank test was performed to test the difference in survival between groups. Multivariate regression analyses of survival data were conducted using the Cox proportional hazards model. RESULTS: Median OS and DFS for all patients were 8.5 and 4.7 years, respectively. Pathologic characteristics that influence OS and DFS were sarcomatous overgrowth (SO), myometrial invasion (MI), lymphovascular invasion (LVI), tumor size, number of mitosis, estrogen receptor, progesterone receptor, International Federation of Gynecology and Obstetrics (FIGO) stage, age, and resection status. Median OS for adenosarcoma patients with SO was 5.2 versus 14.5 years for patients without SO (P < 0.0001). Median OS for adenosarcoma patients with MI was 5.8 years versus not reached for patients without MI (P = 0.0005). Median OS for adenosarcoma patients with LVI was 1.0 versus 8.9 years for patients without LVI (P = 0.0021). On Cox analysis for OS and DFS and LRFS, only SO, MI, LVI, age, resection status, and FIGO stage remained significant. There was no difference in OS or LRFS for adjuvant radiation versus no adjuvant radiation (P = 0.17, P = 0.076). CONCLUSIONS: This study highlights the importance of LVI as a prognostic factor and confirms the prognostic significance of SO, MI, age, resection status, and FIGO stage for adenosarcoma. Furthermore, this study suggests that there is no additional benefit to adjuvant radiation. The standard-of-care treatment for adenosarcoma should remain total abdominal hysterectomy bilateral salpingo-oophorectomy +/- lymphadenectomy and no adjuvant radiation.

Efficacy of Hyperthermic Intraperitoneal Chemotherapy and Cytoreductive Surgery in the Treatment of Recurrent Uterine Sarcoma.

OBJECTIVE: Uterine sarcomas (USs) are characterized by poor response to systemic chemotherapy and high recurrence rates. This study evaluates whether the use of cytoreductive surgery with hyperthermic intraperitoneal chemotherapy (HIPEC) confers survival benefit in comparison with conventional treatment modalities in patients with recurrent US. METHODS/MATERIALS: A retrospective analysis of patients with recurrent US at a single institution for an 11-year study period was performed. All women with a pathologic diagnosis of leiomyosarcoma, adenosarcoma, endometrial stromal sarcoma, or undifferentiated US were identified. Overall and disease-free survival was estimated using Kaplan-Meier method. Comparisons between the study groups were performed with the log-rank test and Cox regression. RESULTS: A total of 26 patients were identified. Five patients received chemotherapy and/or radiotherapy without surgical intervention, 14 patients underwent surgery alone or a combination of surgery and adjuvant systemic chemotherapy, and 7 patients received cytoreductive surgery with HIPEC. There was no treatment-related mortality in any group, and only 1 patient had grade III-IV surgical complications. Median disease-free survival was 2.4 months for patients with nonsurgical treatments, 5.3 months for patients treated with conventional surgery, and 11.3 months for patients treated with HIPEC. Median overall survival was 35.9 months for patients treated with conventional surgery and 43.8 months for patients treated with HIPEC. CONCLUSIONS: Our study is the first to compare survival outcomes of HIPEC versus conventional therapies for recurrent US and is suggestive of treatment benefit. Further studies with more patients and longer follow-up to evaluate the role of HIPEC in management of this disease are warranted.

Survival of women with Mullerian adenosarcoma: A National Cancer Data Base study.

OBJECTIVE: To determine overall survival (OS) and factors associated with OS of women with Mullerian adenosarcoma. METHODS: Women with adenosarcoma of the uterus, cervix or ovary (n=2205) were identified from the 1998-2011 National Cancer Data Base. Kaplan-Meier and multivariate Cox proportional-hazards survival analyses were performed to test for associations of potential explanatory variables with OS. A subset analysis of women with uterine adenosarcoma was also performed. Analyzed confounders included age, insurance status, income, race, surgical margin status, nodal and distant metastasis, surgical procedure type, and treatment with radiation and/or chemotherapy. RESULTS: Primary sites were uterus (n=1884), cervix (n=229) and ovary (n=92), representing 0.43% of uterine, 0.16% of cervical, and 0.04% of ovarian cancers in the NCDB. Only 36/1176 (3.1%) and 2.5% (33/1,342) had nodal and/or distant metastasis, respectively, at diagnosis. Distant metastasis, positive surgical margin, increased age, higher composite comorbidity score and adjuvant radiotherapy were independently associated with decreased OS. Primary site, lymph node status, surgical procedure, chemotherapy use, race, insurance status and income quartiles were not significantly associated with OS. Each 1cm increase in tumor size was associated with increased hazard for death (HR (95% CI) 1.06 (1.01-1.12), p=0.018) among women with uterine adenosarcoma. CONCLUSION: Complete surgical resection remains the only treatment with well-evidenced OS benefit among women with Mullerian adenosarcoma. Early surgical resection may increase survival of Mullerian adenosarcoma.

Uterine Adenosarcoma: a Review.

Adenosarcomas are rare malignancies of the female genital tract, accounting for approximately 5 % of uterine sarcomas. Occasionally, adenosarcoma occurs in the ovaries or in extra-uterine tissue, which may be related to endometriosis. These tumors are characterized by benign epithelial elements and a malignant mesenchymal component. Pathologic diagnosis is dependent on the identification of the characteristic morphologic features. The most common immunohistochemical markers for adenosarcoma are CD10 and WT1, but these are not specific. The most frequent presenting symptom is abnormal uterine bleeding. The majority of patients present with stage I disease, with a 5-year overall survival of 60 to 80 %. Survival is influenced by the presence of myometrial invasion, sarcomatous overgrowth, lymphovascular invasion, necrosis, and the presence of heterologous elements including rhabdomyoblastic differentiation. Patients with sarcomatous overgrowth have significantly increased risk of recurrence 23 versus 77 % and decreased 5-year overall survival 50 to 60 %. Standard of care treatment is total hysterectomy with bilateral salpingo-oophorectomy without lymphadenectomy, as the incidence of lymph node metastasis is rare. Retrospective data does not support the use of adjuvant pelvic radiotherapy in uterine adenosarcomas as no survival benefit is seen. Insufficient data exists to recommend routinely neoadjuvant or adjuvant chemotherapy for uterine adenosarcomas. Limited evidence exists for the role of hormonal therapy in uterine adenosarcomas. The PIK3/AKT/PTEN pathway is mutated in ∼70 % of adenosarcomas, and this may represent a possible therapeutic target. This article reviews the current state of knowledge concerning uterine adenosarcoma and discusses the management of this rare tumor.

Uterine adenosarcoma: an analysis on management, outcomes, and risk factors for recurrence.

OBJECTIVES: Uterine adenosarcoma is a rare malignancy with little data on optimal management. We aimed to clarify the impact of adjuvant therapy in patients with uterine adenosarcoma and identify risk factors for recurrence and death. METHODS: We performed a retrospective review of patients undergoing primary evaluation and treatment for uterine adenosarcoma at a single institution from July 1982 through December 2011. Univariate and multivariate analyses were used to identify prognostic factors for progression-free survival (PFS) and overall survival (OS). RESULTS: We identified 100 patients with uterine adenosarcoma, and 74 patients met the inclusion criteria. On multivariate analysis, sarcomatous overgrowth (SO) and lymphovascular space invasion (LVSI) were predictors of worse PFS and OS. Median PFS and OS were 29.4 and 55.4 months for patients with SO, compared to 105.9 and 112.4 months for patients without SO (PFS HR 2.58, 95% CI 1.37-4.84, p=0.003; OS HR 2.45, 95% CI 1.26-4.76, p=0.008). Among patients with stage I disease, 17 of 22 patients (77%) with SO and 8 of 37 patients (22%) without SO had a recurrence (p<0.001). Among patients with stage I disease with SO, adjuvant therapy appeared to be associated with longer PFS and OS, but these differences were not statistically significant (PFS, 46.7 vs. 29.4 months, p=0.28; OS, 97.3 vs. 55.4 months, p=0.18). CONCLUSION: In patients with uterine adenosarcoma, the presence of SO or LVSI confers a higher risk of recurrence. We did not identify an optimal treatment strategy for patients with SO, but adjuvant therapy may be associated with prolonged PFS.

Gynecologic Cancer InterGroup (GCIG) consensus review for mullerian adenosarcoma of the female genital tract.

Mullerian adenosarcomas of the female genital tract are rare malignancies, originally described in the uterus, the most common site of origin, but they may also arise in extrauterine locations. Uterine adenosarcomas make up 5% of uterine sarcomas and tend to occur in postmenopausal women. They are usually low-grade tumors and are characterized by a benign epithelial component with a malignant mesenchymal component, which is typically a low-grade endometrial stromal sarcoma but can also be a high-grade sarcoma. Tumors that exhibit a high-grade sarcomatous overgrowth have a worse outcome. Adenosarcomas have been described as being midway along the spectrum between benign adenofibromas and carcinosarcomas. They generally have a good prognosis with the exception of deeply invasive tumors or those with high-grade sarcomatous overgrowth. Extrauterine adenosarcomas also have a higher risk for recurrence. In view of their rarity, there have not been any clinical trials in mullerian adenosarcomas and relatively little research. This article reviews the current knowledge and provides recommendation for the management of mullerian adenosarcomas.

Müllerian adenosarcoma: a review of cases and literature.

OBJECTIVE: Mullerian adenosarcoma usually originates in the endometrium and grows as a polypoid mass in post-menopausal women presenting as abnormal vaginal bleeding. This report reviewed Miillerian adenosarcoma cases to clarify the clinical and pathologic characteristics. MATERIALS AND METHODS: Fifteen cases ofMiillerian adenosarcoma in two medical centers covering a 15-year period were reviewed. Their clinical characteristics, pathologic findings, treatment, and outcomes were compared. RESULTS: Of the 15 cases, three originated from the endometrium, six arose from uterine adenomyosis, three from the adnexa, and three from the cervix. There was only one post-menopausal case. One case was of breast cancer with tamoxifen (TMX) therapy. There were four Miillerian adenosarcoma with sarcomatous overgrowth (MASO) cases, three of which died within one year after surgery. Only the focal MASO case survived. CONCLUSION: The rare variant of MASO is very aggressive and associated with poor prognosis.

Management of uterine adenosarcomas with and without sarcomatous overgrowth.

OBJECTIVES: Uterine adenosarcomas (AS) are rare tumors composed of malignant stromal and benign epithelial components. We sought to evaluate the role of primary surgery, adjuvant treatments, and salvage therapies for patients with uterine adenosarcomas. METHODS: We identified all patients diagnosed with AS from 1990 to 2009 at our institution. Patient demographics, surgical procedures, sites of metastatic disease, and histologic features (e.g., presence of sarcomatous overgrowth, and heterologous elements) were collected. Treatment regimens and survival outcomes were evaluated. RESULTS: Thirty-one patients were evaluable for this study: 19 (61%) received up-front treatment at our institution and 12 (39%) received treatment for recurrent disease. Most of the up-front treated patients (15, 79%) were diagnosed with stage I disease and underwent hysterectomy (100%) with bilateral salpingo-oophorectomy (84%). Of the 19 patients treated at our institution from time of initial diagnosis, 5 (26%) patients recurred (median follow-up, 72.9 months; range, 3-154). In 5 patients with sarcomatous overgrowth (AS+SO), the 2-year progression-free and overall survival rates were both 20% versus 100% for 14 patients without sarcomatous overgrowth. Responses to systemic treatment of measurable disease were observed in patients with and without sarcomatous overgrowth, but no optimal treatment strategy could be identified for either groups. CONCLUSIONS: Unlike AS without sarcomatous overgrowth, AS+SO is an aggressive disease with a high recurrence rate. In our series, no optimal adjuvant or systemic treatment strategy was identifiable but standard sarcoma chemotherapy regimens appear to have efficacy in both AS and AS+SO.

Uterine adenosarcomas: a dual-institution update on staging, prognosis and survival.

OBJECTIVE: Uterine adenosarcomas (AS) are rare tumors thought to have a favorable prognosis. The aim of this study was to evaluate clinicopathological characteristics and treatment outcome in women with uterine AS. METHODS: Patients with uterine AS were identified from the institutional databases at two regional cancer centers, Princess Margaret Hospital, Toronto and Vancouver General Hospital. All cases underwent specialist pathological review and were re-staged according to FIGO criteria (2009). Patient demographics, treatment data and outcomes were evaluated. RESULTS: Between 1984 and 2010, 64 patients with confirmed AS were identified: 30 exhibited sarcomatous overgrowth (AS+SO). 47 patients presented with stage I disease: 27 IA and 18 IB. 57 of the 58 patients with known surgical management underwent hysterectomy: 55 having bilateral salpingo-oophorectomy, 12 having lymph node dissection. 14 patients received adjuvant treatment: 10 radiotherapy, 3 chemotherapy and 1 both. Sixteen of the 45 patients (35.6%) with follow-up recurred; median time to recurrence 21.2 months, range 2.1-87.8 months. Recurrence was associated with myometrial invasion (p=0.05). Two of the 10 women (20%) with AS+SO receiving adjuvant treatment recurred compared to 9 of the 14 (64%) who did not. One of the 5 women (20%) with stage IB disease who received adjuvant treatment recurred (20%) compared to 6 of the 7 (85.6%) who did not. CONCLUSIONS: Long term surveillance is required given the variable time to recurrence. For those with AS+SO and myometrial invasion adjuvant treatment should be considered and further investigation of adjuvant strategies is warranted.

Müllerian adenosarcoma with a neuroectodermal component associated with an endometriotic cyst of the ovary: a case report.

Reported here is a case of Müllerian adenosarcoma of the ovary which contained a primitive neuroectodermal tissue component within the stroma. The adenosarcoma coexisted with clear cell adenocarcinoma in an endometriotic cyst. The patient was a 33-year-old woman with a large unilocular endometriotic cyst of the right ovary. On the internal wall of the cyst, both a plaque-like protrusion with a papillary surface and a dome-like mural nodule were noted. The former exhibited features of Müllerian adenosarcoma, and the latter showed those of clear cell adenocarcinoma. In the deeper portion of adenosarcoma, teratoma-like tissue which contained various tissue components including primitive neuroectodermal tissue was found. The presence of primitive neuroectodermal tissue in the stroma of adenosarcoma suggested the diagnosis of 'adenosarcoma with neuroectodermal differentiation' ('teratoid adenosarcoma'), although the possibility of the incidental occurrence of an immature teratoma could not be completely excluded.

Uterine inversion in association with uterine sarcoma: a case report with MRI findings and review of the literature.

Non-puerperal uterine inversion due to uterine sarcomas represents a very rare event with no reliable estimate of frequency in the literature. Clinically, the diagnosis of inversion may be difficult, as far as imaging procedures are concerned, although ultrasonography may prove to be useful. However, some characteristics such as the indentation of the fundic area and a depressed longitudinal groove extending from the uterus to the center of the inverted portion are difficult to recognize. Moreover, there is no specific computed tomography feature accurate enough to aid in the differential diagnosis. Here, we report a case of uterine inversion due to Müllerian uterine adenosarcoma whose preoperative workup and diagnosis took advantage of the application of magnetic resonance imaging.

The first successful treatment and genetic sequencing of primary hepatic adenosarcoma with sarcomatous overgrowth: a case report.

Adenosarcoma is a rare type of tumor with a mixture of epithelial and stromal components and often occurs in the female reproductive system. Primary hepatic adenosarcoma (PHAS) is extremely rare, with only two cases reported so far. Both patients had poor outcomes. Here, we report the case of a 36-year-old man with pain under the xiphoid process who was diagnosed with a bile duct tumor. He was treated with adjuvant radiotherapy when surgery was performed on him. Pathologically, the tumor contained benign epithelial tissue, and the submucosa of the bile duct in the liver showed infiltrating growth of spindle cell components. The cells were dense, mildly heterotypic, and occasionally mitotic, and the patient was diagnosed with PHAS. Whole-exome sequencing results showed that a total of 12 mutations were shared by the two tissues. The patient received adjuvant radiotherapy and he was tumor-free until 31 months postoperatively. This case will provide some references of the disease to other researchers.

Uterine müllerian adenosarcoma with sarcomatous overgrowth and lung metastasis in a 25-year-old woman.

Uterine müllerian adenosarcoma with sarcomatous overgrowth (MASO), uncommon in premenopausal women, is a rare variant of uterine adenosarcomas characterized by a sarcomatous portion constituting >25% of the tumor. Uterine MASO often appears as a benign, protruding cervical polyp. However, in contrast to typical müllerian adenosarcomas (MAs), MASO is a highly aggressive tumor, frequently associated with a fatal outcome. Though very rare in premenopausal women, because of the high aggressiveness and malignant potential, uterine MASO should be considered, even in women of a young age with benign-appearing polypoid masses, and treated aggressively at the time of initial diagnosis without delay. We present herein a case of uterine MASO in a 25-year-old woman with lung metastasis who was lost to follow-up for one month after the initial diagnosis had been established.

Mixed Endometrioid Adenocarcinoma and Müllerian Adenosarcoma of the Uterus and Ovary: Clinicopathologic Characterization With Emphasis on its Distinction From Carcinosarcoma.

Mullerian adenosarcoma is a biphasic neoplasm composed of benign or atypical Müllerian epithelium and a malignant mesenchymal component that is usually, but not always, of low grade. Focal architectural or cytologic atypia of the epithelial component resembling atypical hyperplasia may uncommonly be present and foci of adenocarcinoma have been rarely reported. Whether the coexistence of these 2 tumor components is a result of independent primaries (collision tumor), adenocarcinoma arising from the epithelial component of the adenosarcoma, an unusual form of carcinosarcoma or some other mechanism is uncertain. To establish the diagnostic criteria and clinical significance of the coexistence of adenocarcinoma in close association with Müllerian adenosarcoma, we conducted a multi-institutional study of these rare tumors. Twenty-six patients were identified with "mixed" adenosarcoma and adenocarcinoma; they ranged in age from 43 to 87 years (median: 66 y). Tumors occurred in the uterine corpus (n=22), ovary (n=2), and the pelvis (n=2). All but 6 had International Federation of Gynecology and Obstetrics (FIGO) stage I disease. All extrauterine tumors were associated with endometriosis. The tumor size ranged from 2 to 25 cm (median: 7.9 cm). The sarcomatous component was of low grade in 18 and high grade in 8 (the majority demonstrating rhabdomyoblastic differentiation); 9 had stromal overgrowth. Twenty-five carcinomas were endometrioid in type (23 FIGO grade 1; 3 FIGO grade 2) and 1 carcinoma was dedifferentiated with FIGO grade 1 endometrioid adenocarcinoma component; 33% of the uterine neoplasms were associated with adjacent endometrial hyperplasia. Next-generation sequencing in 2 tumors identified similar molecular abnormalities in the sarcomatous and carcinomatous components supporting a clonal relationship. Of 10 patients with available follow-up (median: 18 mo), 8 had no evidence of disease and 2 died of recurrent sarcoma at 7 and 8 months. Endometrioid adenocarcinomas that arise in close spatial association with Müllerian adenosarcoma appear to be clonally related to the sarcoma. Unlike carcinosarcomas, these tumors are usually early stage at presentation. The prognosis appears to be driven by the sarcomatous component. These tumors should be distinguished from carcinosarcomas, dedifferentiated endometrial carcinomas, and corded and hyalinized endometrioid carcinomas.

Hepatocellular carcinoma and hepatic adenocarcinosarcoma in a patient with hepatitis B virus-related cirrhosis.

The authors present the case of a 48-year-old man with hepatitis B cirrhosis, who developed two primary malignant liver tumors that were morphologically distinct from each other. The first tumor was a hepatocellular carcinoma and the second tumor, detected 17 months later was a hepatic carcinosarcoma with cholangiocarcinomatous and sarcomatous components, without any hepatocellular carcinoma component. Clonality studies using microsatellite-based loss of heterozygosity (LOH) demonstrated different LOH patterns existed between the hepatocellular carcinoma and the hepatic carcinosarcoma, indicative of different clonal origins. The authors discuss the histogenesis, histopathologic diagnosis, and clinical behavior of hepatic carcinosarcoma.

Mullerian adenosarcoma of the uterus: a rare neoplasm with a need for onco-fertility.

A 23-year-old nulliparous woman re-presented with menorrhagia and intermenstrual bleeding two years after her first presentation with a similar history. Her initial symptoms were thought to be due to a removed fibroid polyp with histological confirmation. However at the second presentation, following a polypectomy, a diagnosis of low-grade mullerian adenocarcinoma of the uterine body was made. She had total abdominal hysterectomy and pelvic lymph node dissection, peritoneal fluid for cytology with conservation of ovaries to conserve her fertility. No residual tumour was found and lymph nodes were negative. She remains well under clinical surveillance in a multidisciplinary team setting. Different management options that have been used in past reports have been examined and also fertility sparing surgical techniques available for use in successful management of gynaecological cancer are also being explored to shed more light on potential surgical techniques that may be used in treating such rare tumours, particularly in women wishing to retain their fertility.