Presence Of Heterologous Elements Research Articles

Müllerian adenosarcoma with sarcomatous overgrowth and heterologous elements: A case report and literature review

Uterine mullerian adenosarcoma (MA) is a rare biphasic tumour that accounts for less than 0.5% of uterine neoplasms. The age range of presentation is wide, with the median age in the 5th decade of life. It usually has a good prognosis; however, it worsens when it presents with sarcomatous overgrowth, heterologous elements or infiltrates the myometrium.We report the case of a 63-year-old woman presenting with abnormal vaginal bleeding and a sensation of solid material coming out of the cervical canal who was diagnosed with mullerian adenosarcoma with sarcomatous overgrowth (MASO) and presence of heterologous elements after performing a mass biopsy and subsequent hysterectomy.We reviewed the literature, focusing especially on the differential diagnoses to be evaluated, as well as the differences in prognosis and treatment according to whether or not they present histologic features of poor prognosis.

Postmenopausal Ovarian Sertoli-Leydig Tumours: Case Report

Sertoli and Leydig cell tumours are rare secretory tumours of the mesenchyme and sex cords. Diagnostic certainty is histological after surgery and there is no specific ultrasound sign despite the strong clinical presumption. The prognosis depends on the degree of cellular differentiation and the presence of heterologous elements. We report on the occurrence of a sertoli-leydig tumour in a 50-year-old postmenopausal woman. Poorly differentiated forms of Sertoli-Leydig tumours have a significant malignant potential. Treatment is surgical, with chemotherapy providing an interesting adjuvant. The prognosis after surgery is dominated by recurrence.

Epithelial to mesenchymal transition (EMT) in metaplastic breast cancer and phyllodes breast tumors.

Epithelial-mesenchymal transition (EMT), a transdifferentiation program whereby epithelial cells acquire mesenchymal phenotype, is essential during embryonic development. EMT has also been implicated in cancer progression by conferring migratory and metastatic potential, as well as cell plasticity and stem cell like traits, to cancer cells. Metaplastic breast carcinoma (MBC) is a rare aggressive type of breast cancer characterized by the presence of heterologous elements, typically by the existence of epithelial and mesenchymal components. Phyllodes tumors (PTs) are uncommon fibroepithelial neoplasms consisting of epithelial and mesenchymal elements. Although various hypotheses have been proposed on the pathogenesis of these biphasic tumors, there is growing evidence supporting the theory that PTs and MBC could both correlate with cancer related EMT. This review summarizes the existing literature on the emerging role of EMT in the pathogenesis of MBC and PTs. Both malignant PTs and MBC are characterized by poor prognosis. Therefore, several anti-EMT targeting strategies such as blocking upstream signaling pathways, targeting the molecular drivers of EMT and targeting mesenchymal cells and the extracellular matrix, could potentially represent a promising therapeutic approach for patients suffering from these aggressive neoplasms.

Malignant phyllodes tumor with heterologous sarcomatous element: A rare case report

Phyllodes tumors being a rare neoplasm of the breast attributes only 0.3% to 1% of all breast tumors, with an annual incidence of about 2 per million women. Malignant differentiation of phyllodes tumors can occur, resulting in cases of extremely rare heterologous sarcomatous differentiation. We present a rare case of a 63-year-old women with features of malignant phyllodes with sarcomatous (chondrosarcomatous) differentiation.Morphologically, phyllodes tumors are categorized as benign, borderline and malignant based on the scope of stromal atypia, mitotic activity, stromal overgrowth, and the tumor margins. The presence of heterologous elements in a phyllodes directly upgrades it to a malignant category. A close follow-up with a CT scan is of utmost need to detect the recurrence and metastasis to the earliest.

Malignant phyllodes tumors of the breast: the malignancy grading and associations with prognosis

This study aimed to correlate clinicopathological parameters with survival outcomes in a cohort of patients diagnosed with malignant phyllodes tumors (MPTs). We also analyzed the malignancy grade of MPTs and investigated the prognostic significance of the malignancy grading system. Clinicopathological parameters, malignancy grades, and clinical follow-up data of 188 women diagnosed with MPTs in a single-institution were analyzed. MPTs of the breast were grouped according to stromal atypia, stromal overgrowth, mitotic count, tumor differentiation, and necrosis. A Fleiss' kappa statistic was calculated to test the agreement between the pathologists for the grading of MPTs. Disease-free survival (DFS), distant metastasis-free survival (DMFS) and overall survival (OS) were estimated using the Kaplan-Meier method and compared between groups using the log-rank test. Cox regression was carried out to identify factors predictive of locoregional recurrence (LRR), distant metastasis (DM) and death. A total of 188 MPTs were classified according to the malignancy grading system: 88 (46.8%) as low grade, 77 (41%) as an intermediate grade, and 23 (12.2%) as high grade. Excellent agreement between pathologists for the grading of MPTs (Fleiss' kappa 0.807). In our study population, the occurrence of DM and death were associated with the malignancy grade of MPTs (P < 0.001). Based on the DFS curves, the presence of heterologous elements (P = 0.025) and younger age (P = 0.014) were independent prognostic indicators. Additionally, the malignancy grade retained independent prognostic significance for predicting DMFS and OS (P < 0.001 and P = 0.009). Higher malignancy grade, presence of heterologous elements, younger age, larger tumor size, and recent rapid tumor growth are poor prognostic factors for MPTs of the breast. The malignancy grading system may be generalized in the future.

Sarcomatoid Chromophobe Renal Cell Carcinoma with Heterologous Elements: A Rare Form of an Aggressive Tumor

Abstract Introduction/Objective Chromophore renal cell carcinoma (ChRCC) accounts for approximately 4-10% of renal cell carcinomas. Compared with other subtypes of renal cell carcinoma, ChRCC generally has a more favorable outcome; however, sarcomatoid transformation is greatest in ChRCC. We report a case of ChRCC with extensive sarcomatoid transformation, which included the heterologous elements of osteosarcoma and chondrosarcoma. The presence of heterologous elements is distinctively rare, with only twelve previous cases in the English literature. Methods/Case Report A 64-year-old man presented with left flank pain and night sweats. The abdominal MRI showed a large exophytic hypervascular mass in the left kidney with metastasis to the left para-aortic lymph nodes. The patient underwent radical nephrectomy. Two months after the surgery, the patient was found to have extensive bony metastases. On gross examination, the nephrectomy specimen showed an 18 cm tumor replacing almost the entire kidney. The cut surface was white to tan with areas of hemorrhage and necrosis. Microscopic examination revealed a predominantly sarcomatoid ChRCC (nearly 80% of the tumor) with heterologous chondrosarcoma and osteosarcoma. The tumor extended into the perinephric adipose tissue and the adrenal gland. Multiple para-aortic lymph nodes contained sarcomatoid tumor with heterologous elements. Results (if a Case Study enter NA) NA. Conclusion ChRCC is frequently considered a more indolent renal cell carcinoma. It is important to recognize its higher propensity for sarcomatoid transformation. The presence of heterologous elements is exceptionally uncommon. Whether this correlates with a more aggressive tumor is not known because of the paucity of such cases.

Phyllodes Tumors: A Scoping Review of the Literature.

Phyllodes tumors are rare tumors of the breast, with most literature being retrospective with limited guidelines on the management of these patients. Scoping review was performed to highlight themes and gaps in the available literature. A scoping review of the literature was performed as per PRISMA-ScR guidelines with titles, abstracts, and full texts reviewed in duplicate. Data were abstracted and summarized in categories of diagnostics, surgical management, adjuvant therapies, treatment of recurrence, and surveillance. Quality assessment was performed for each paper. The search identified 4498 references, 434 full-text papers were reviewed, and 183 papers were included. Ultrasound, magnetic resonance imaging, and core needle biopsy are valuable preoperative diagnostics tools. Pathology reporting should include stromal overgrowth, stromal cellularity, nuclear atypia, mitotic rate, borders, and presence of heterologous elements. Ki67 may have a role in grading and prognosticating. Breast conservation is safe in all grades of phyllodes but may be associated with increased local recurrence in malignant phyllodes. Surgical margins should depend on grade. Axillary node positivity rate is very low, even with clinically enlarged lymph nodes. Adjuvant radiation is a useful tool to decrease local recurrence in malignant phyllodes tumors, tumors > 5 cm, age < 45 years, close margins, and breast conservation. There is no evidence supporting adjuvant chemotherapy. Recurrence can be managed with repeat wide excision; however, mastectomy is associated with lower re-recurrence. Surveillance protocols are variable in the literature. There is heterogeneity in the literature on phyllodes tumors. Consensus guidelines based on the literature will help provide evidence-based care.

An Infrequent Adenosarcoma Mimicking other Uterine Rhabdoid Tumours

Uterine sarcomas are relatively rare and account for only 1-3% of all malignancies in the Female Genital Tract (FGT). Adenosarcomas of the uterus are uncommon accounting for only 5% of uterine sarcomas. They are considered less recurrent and less metastatic. However, those with sarcomatous overgrowth and heterologous differentiation are very rare, considered aggressive and need to be differentiated histologically from other sarcomas that mimic the same clinically and histologically. In the index case, a 50-year-old female presented with symptoms of pain in the abdomen and bleeding per vagina. Magnetic Resonance Imaging (MRI) showed a large polypoid solid mass in the endometrium, suggestive of a sarcoma. Positron Emission Tomography (PET) scan showed an Fluorodeoxyglucose (FDG) avid lesion in the endometrial cavity (SUV max-13.33). Total abdominal hysterectomy and salpingooophorectomy was performed. Histology showed a biphasic tumour (benign glands and malignant mesenchymal component) with rhabdomyoblastic differentiation and sarcomatous overgrowth. Presence of rhabdoid cells raises the possibility of other sarcomas with these features making the diagnosis challenging. Immunohistochemistry (IHC) confirmed it to be a high grade adenosarcoma with sarcomatous overgrowth. Desmin positivity was noted in the rhabdoid cells. Majority cases of adenosarcoma are low grade, the index case was however high grade with presence of heterologous elements and sarcomatous overgrowth. Neither radiology nor clinical features can reliably differentiate adenosarcomas from other uterine sarcomas. A definitive diagnosis can be made by understanding the overlapping morphological features and correlate it with IHC to reliably differentiate it with other similar looking uterine sarcomas.

Uterine and Cervical Adenosarcoma: A Retrospective Study of Overall Oncologic Outcomes and Fertility Preservation in Early-Stage Disease

The management of adenosarcoma is based on the limited available data. This study aimed to explore the characteristics and oncologic outcomes of patients with uterine and cervical adenosarcoma. A total of 21 and 32 cases of cervical and uterine adenosarcoma, respectively, were retrospectively reviewed in Peking Union Medical College Hospital between April 2006 and March 2019. The median follow-up time was 37.5 months (range, 1-153 months). The disease progression rate (DPR) was significantly higher in patients with uterine adenosarcoma compared with those with cervical adenosarcoma (28.1% vs. 4.8%). The curve of progression-free survival significantly differed. For those with cervical adenosarcoma, the presence of a tumor stalk was a protective factor, whereas heterologous elements (HE) presented a risk factor for disease progression. For those with uterine adenosarcoma, the presence of a tumor stalk was an independent protective factor, whereas lymphovascular space invasion (LVSI) was an independent risk factor for disease progression. Moreover, HE was an independent risk factor for mortality. Fertility-sparing surgery (FSS) was performed in four and five patients with cervical and uterine adenosarcoma, respectively. Regarding FSS, combined with cases in previous studies, the DPR of patients with uterine adenosarcoma was relatively higher compared with those with cervical adenosarcoma. We found that cervical adenosarcoma had a better prognosis than uterine adenosarcoma. The presence of a tumor stalk was a protective factor, whereas HE and LVSI were risk factors for prognosis. For those with uterine adenosarcoma, if FSS was administered, robust evaluation would be necessary. The small sample size limits the ability to make any strong conclusions about FSS. Uterine cervical adenosarcoma had a better prognosis than uterine adenosarcoma. For patients with cervical adenosarcoma, the presence of a tumor stalk was a protective factor and the presence of heterologous elements (HE) was a risk factor for disease progression. For those with uterine adenosarcoma, the presence of a tumor stalk was a protective factor and lymphovascular space invasion was a risk factor for disease progression. Moreover, HE was a risk factor for mortality. Regarding fertility-sparing surgery (FSS), the disease progression rate was higher in patients with uterine adenosarcoma compared with those with cervical adenosarcoma. For patients with uterine adenosarcoma, if FSS was administered, hysteroscopy and robust imaging evaluation would be necessary.

Sertoli–Leydig Cell Tumour with Heterologous Element with Rare Mitotic Figure Associated with Multinodular Goitre: A Rare Case of Sex Cord Stromal Tumour of Ovary with Rare Association

Sertoli–Leydig cell tumours are uncommon sex cord stromal tumours of the ovary that often present diagnostic confusion due to the various morphological and histopathological patterns that may be assumed by the tumour. A 28-year-old female presented with abnormal uterine bleeding and thyromegaly is found to have right adnexal mass with elevated serum testosterone. The mass was diagnosed as a Sertoli–Leydig cell tumour, intermediate differentiation with heterologous elements and focal anaplasia. She was treated with surgical resection followed by adjuvant chemotherapy. Definitive diagnosis is made by histopathological study with immunohistochemistry. Prognosis is related to the degree of cellular differentiation and to the presence of heterologous elements with stage of disease at the time of diagnosis.

The Impact on Survival of an Extensive Sex Cord-like Component in Mullerian Adenosarcomas: A Study Comprising 6 Cases.

Mullerian adenosarcomas are uncommon tumors of the female genital tract characterized by a synchronous proliferation of benign glands and sarcomatous stroma. In general, uterine Mullerian adenosarcomas are associated with a low risk of recurrence. The presence of "stromal overgrowth" (SO), historically defined by an estimate of the volume of sarcoma growing independently of epithelium, is associated with deep myometrial invasion, presence of heterologous elements, and poor outcomes. Very rarely, the stromal component can harbor foci resembling ovarian sex cord tumors (FROSCT). The aim of this study was to determine whether the presence of an extensive FROSCT component in Mullerian adenosarcomas has an impact on survival, akin to more typical types of SO. Six patients were included in this study. Age ranged from 39 to 71 yr. Five patients presented with uterine lesions (4 intracavitary, 1 isthmic), and 1 was located in the ovary. Tumors ranging in size from 2.5 to 19 cm were all diagnosed as Stage I. Morphologically, all had prominent FROSCT-like components that comprised 60% to 90% of tumor volume. Immunohistochemically, the FROSCT component was positive for CAM 5.2, vimentin, WT1, CD56, α-inhibin, calretinin, androgen and progesterone receptors, α-actin, and desmin. All patients are alive without disease at 26 to 102 mo. Compared with adenosarcomas with typical forms of SO, FROSCT overgrowth is low grade and not associated with recurrence or metastasis in this small series. Therefore, Mullerian adenosarcoma with extensive FROSCT should not be equated with SO.

Uterine sarcomas.

Uterine sarcomas.

930P - A Retrospective Single Institution Study Evaluating Clinical Outcome and Prognostic Markers for Endometrial and Ovarian Carcinosarcomas (Cs)

ABSTRACT Aim: To assess whether: (1) clinical outcomes for ovarian vs. endometrial CS were comparable (2) the epithelial histological subtype or presence of heterologous elements in the mesenchymal component impacted overall survival (OS) (3) a personal or family history (FHx) of cancer was associated with OS. Methods: Women treated at University College London Hospital Gynae-oncology Unit between April 2002 – 2011 were identified using electronic records. Patient clinical data and full histological reports were collected retrospectively from the medical records. Results: 82 patients (72 endometrial; 10 ovarian) with a median age of 67 years were identified. 22% had a prior cancer history and 3.6% had previously received abdominal/pelvic radiotherapy (RT). Median OS was 26.7 months. Analysis for OS according to baseline characteristics OS #events/n HR(95% CI) p-value Original Tumour site Endometrial Ovarian 33/72 6/10 1.0 1.33 (0.56 -3.17) 0.5 FIGO Stage I/II III/IV 10/37 22/33 1.0 3.22 (1.52, 6.82) 0.001 Epithelial type Poorly/un-differentiated Other 15/39 16/31 1.0 1.55 (0.75,3.18) 0.23 Cancer History No Yes 22/56 10/18 1.00 1.23 (0.59, 2.55) 0.58 Cancer FHx No Yes 26/56 7/15 1.00 1.08 (0.47, 2.49) 0.86 All ovarian CS patients had surgical resection (SR), 90% completed 6 cycles of postoperative carboplatin/paclitaxel chemotherapy (CT) with 20% complete responses (CR) and 40% partial responses (PR). 88% of endometrial CS patients were treated with SR, CT +/- RT. The response rates (RR) after CT were 70% CR and 9% PR. Neither the epithelial subtypes, nor the presence of a heterologous component showed statistically significant difference for OS. Conclusions: Clinical outcome for ovarian (12%) vs. endometrial CS were comparable in regards to OS. FIGO stage was the only statistically significant prognostic factor. There was a trend for worse OS in CS with poorly/un- differentiated adenocarcinoma and/or absence of heterologous components although statistically not significant in this cohort. This warrants further clinical evaluation in a larger study. Disclosure: All authors have declared no conflicts of interest.

An Abnormal Cervicovaginal Cytology Test in Malignant Mixed Mullerian Uterine Tumor has a Predictive Value of Cervical Involvement Independent of Tumor Stage

Introduction: Malignant Mixed Mullerian Tumor/Carcinosarcoma (MMMT) is a clinically aggressive endometrial malignancy composed of mixture of carcinomatous and sarcomatous elements usually affecting postmenopausal women. As this tumor is relatively rare and morphologically diverse, the findings on cervicovaginal cytology, specifically liquid based “SurePath” cytology samples, are not well described. Here we describe the cytological features and correlation with histopathological and clinical findings of patients with MMMT. Material and Methods: A retrospective search (2001-2012) of patients with MMMT was performed. Available PAP smears, biopsies and surgical excisions were reviewed. Tumor characteristics including tumor size presence of heterologous elements, cervical involvement, lympho-vascular invasion (LVI) and tumor pathology staging were extracted from pathology reports and analyzed. Results: There were a total of 30 MMMT patients with available PAPs preceding surgery. All except two patients (28/30) were African American women with average age of 63.03. Ten patients (33.3%) had negative PAPs, eleven (36.7%) were diagnosed as malignant (carcinoma or adenocarcinoma) and nine (30%) as atypical (AGUS, ASCUS or squamous dysplasia). Patients with positive PAP smears had larger tumors and in almost all (90.9%) of the cases tumor showed cervical stromal involvement, and LVI (90.9%). Group with atypical pap smear result did not show correlation with any tumor characteristics or tumor stage. Negative results on PAP smears were seen more commonly in patients without cervical involvements (80%). Common cytology features were tumor diathesis, glandular atypia, squamous dysplasia, presence of nucleoli, multinuclear and anaplastic cells, while sarcomatous elements were rarely encountered. Conclusions: Two thirds of patients with MMMT had either positive or atypical PAPs prior to biopsy or surgery. Patients with cervical involvement of MMMT were more likely to have positive PAP smear independent of tumor staging.

HPV DNA Testing as Triage for Unsatisfactory Liquid-Based Cervicovaginal Cytology Specimens

Introduction: Malignant Mixed Mullerian Tumor/Carcinosarcoma (MMMT) is a clinically aggressive endometrial malignancy composed of mixture of carcinomatous and sarcomatous elements usually affecting postmenopausal women. As this tumor is relatively rare and morphologically diverse, the findings on cervicovaginal cytology, specifically liquid based “SurePath” cytology samples, are not well described. Here we describe the cytological features and correlation with histopathological and clinical findings of patients with MMMT. Material and Methods: A retrospective search (2001-2012) of patients with MMMT was performed. Available PAP smears, biopsies and surgical excisions were reviewed. Tumor characteristics including tumor size presence of heterologous elements, cervical involvement, lympho-vascular invasion (LVI) and tumor pathology staging were extracted from pathology reports and analyzed. Results: There were a total of 30 MMMT patients with available PAPs preceding surgery. All except two patients (28/30) were African American women with average age of 63.03. Ten patients (33.3%) had negative PAPs, eleven (36.7%) were diagnosed as malignant (carcinoma or adenocarcinoma) and nine (30%) as atypical (AGUS, ASCUS or squamous dysplasia). Patients with positive PAP smears had larger tumors and in almost all (90.9%) of the cases tumor showed cervical stromal involvement, and LVI (90.9%). Group with atypical pap smear result did not show correlation with any tumor characteristics or tumor stage. Negative results on PAP smears were seen more commonly in patients without cervical involvements (80%). Common cytology features were tumor diathesis, glandular atypia, squamous dysplasia, presence of nucleoli, multinuclear and anaplastic cells, while sarcomatous elements were rarely encountered. Conclusions: Two thirds of patients with MMMT had either positive or atypical PAPs prior to biopsy or surgery. Patients with cervical involvement of MMMT were more likely to have positive PAP smear independent of tumor staging.

Mullerian Adenosarcoma of Uterus with Sarcomatous Overgrowth and Heterologous Component Associated with Stromal Deposit in Omentum: A Case Report and Review of the Literature

Background. Mullerian adenosarcoma with sarcomatous overgrowth (MASO) is a very rare variant of uterine sarcomas first described by Clement et al. as early as 1974. The presence of heterologous sarcomatous components is associated with aggressive biological behavior. Case Presentation. This is a case report of a 62-year female (P2 + 0) presenting with postmenopausal vaginal bleeding. Her preoperative USG revealed subserosal fibroid with adherent omentum. She underwent abdominal hysterectomy with bilateral oophorectomy. Histopathological diagnosis of resected specimen was Mullerian adenosarcoma with sarcomatous overgrowth and presence of heterologous elements involving body of the uterus. The whole thickness of the myometrium was involved along with the presence of serosal nodules and omental deposits of sarcomatous component. Conclusion. MA is considered as a low-grade malignant tumor, but MASO is a high-grade tumor frequently associated with invasion and metastasis with poor treatment outcome. Because of its rarity, correct identification of these tumors and distinction from other uterine sarcomas are a challenging job and hence its morphological features merits attention.

Pathologic and molecular features of uterine carcinosarcomas

Uterine carcinosarcomas (UCSs), formerly known as malignant mixed müllerian tumors, are uncommon neoplasias that account for <5% of uterine malignancies. Traditionally, UCSs have been considered a subtype of sarcoma and the staging system and adjuvant oncological treatments used have been similar to those used for high-grade uterine sarcomas. However, there is now enough clinical, pathologic, and biological evidence to consider UCSs more closely related to high-grade endometrial carcinomas. Thus, these tumors should be staged based on the surgicopathologic staging system used for endometrial carcinomas. Morphologically, UCSs are heterogeneous biphasic tumors composed of an admixture of malignant (endometrioid and nonendometrioid) epithelial and (homologous and heterologous) mesenchymal elements in different proportions. UCSs predominantly metastasize as carcinomas and they are associated with a poor prognosis. Although stage is a consistent prognostic factor, the significance of several histopathological features, such as myometrial invasion, lymphovascular space involvement, type of carcinomatous component, extent of the sarcomatous component, and the presence of heterologous elements, remains controversial and probably differs among different stages. Although the diagnosis of UCS is not difficult in most cases, the differential diagnosis may include entities such as undifferentiated or dedifferentiated carcinoma, endometrioid adenocarcinoma with spindle cell elements, sarcomatous overgrowth in a low-grade müllerian adenosarcoma, and pure malignant mesenchymal tumors. Genetic and molecular studies have confirmed the clonal origin of most UCSs and have shown these tumors to be similar to those observed in high-grade/nonendometrioid carcinomas, with p53 mutations being the most common molecular alteration. Finally, from a biological standpoint, the process by which epithelial malignant cells of UCS transdifferentiate to malignant mesenchymal cells could be considered a true example of epithelial mesenchymal transition in human neoplasias.

Immunohistochemical and ultrastructural analysis of a poorly differentiated pediatric age Sertoli–Leydig cell tumor

Ovarian Sertoli–Leydig cell tumors (SLCT) are rare in young women. They are divided into six categories based on the degree of differentiation and the presence of heterologous elements. Less than 15% of these tumors are poorly differentiated. A 14-year-old obese African-American girl presented with amenorrhea, progressive abdominal pain, and increasing abdominal girth. Pelvic CT revealed a 10 × 9 × 9 cm right adnexal mass which was resected successfully. The gross appearance was dark tan and red with central hemorrhage and necrosis. Microscopically, this was poorly differentiated with compact aggregates of moderate size oval to elongated cells separated by zones of edematous stroma containing scattered spindle shape cells. Areas of ill-formed tubules and primitive cords were present. Clusters of Leydig cells were observed. The oval and spindle cells showed multiple mitoses and were diffusely positive for inhibin and patchy but strong positivity for calretinin. Both preoperative and postoperative studies revealed no metastases. Serum α-fetal protein (AFP), androgen, and dihydroepiandrosterone sulfate (DHEA-S) were elevated.

Müllerian Adenosarcoma of the Cervix with Heterologous Elements: Report of a Case and Review of the Literature

Background. Müllerian adenosarcoma (MA) is a rare neoplasm composed of benign epithelial and malignant stromal components. Its location in the cervix and the presence of heterologous elements are extremely infrequent (to our knowledge, only 14 more cases have been reported in the English literature). We describe another case of MA of the uterine cervix with heterologous elements and review the clinical and pathological features of these tumors.Case report. A nulliparous 25-year-old woman was admitted to the hospital because of metrorrhagia and recurrent masses, dependent on the cervix, initially considered endocervical polyps. Microscopically, the last curettage performed revealed a tumor composed of two elements, epithelial and mesenchymal. The epithelial elements were benign endocervical type glands, and the mesenchymal were sarcomatous, containing minor foci of cartilage. A diagnosis of endocervical heterologous adenosarcoma was reached, and a total hysterectomy and bilateral salpingo-oophorectomy were performed. The patient is alive 2 years after the surgical procedure. Neither chemotherapy nor other adyuvant therapies have been administered, and she is clinically free of disease at the moment.Conclusion. Because MA with heterologous elements seems to appear at the earliest stages of the reproductive lifespan in women, commonly with a history of recurrent polyps, and because its malignant potential is uncertain, gynecologists and pathologists should be aware and think about the possibility of this tumor. Long-term follow-up is essential and so is the accumulation of individual cases to provide further experience with these unusual neoplasms.

Uterine and extrauterine Mullerian adenosarcoma: a histopathologic and flow cytometric study

Uterine adenosarcoma is a rare tumor of Mullerian origin and is characterized by a benign glandular component and a sarcomatous usually low-grade, stroma, We examined 11 cases of Mullerian adenosarcoma, eight cervical, two uterine and one of the peritoneum. Tissue protruding from the cervix and an initial diagnosis of a cervical polyp were common findings for most patients with uterine adenosarcoma. On repeated examination these cases were re-evaluated as Mullerian adenosarcomas. Histologic examination revealed benign glands with a sarcomatous stroma, which typically formed periglandular cuffs of increased cellularity. The saromatous stroma was homologous in four cases but contained heterologous elements, such as striated muscle, lipoblasts and cartilage in three cases; in one case a sarcomatous overgrowth of stromal elements was found. The question of a Mullerian adenofibroma vs adenosarcoma was raised in three cases. According to stromal cellularity, stromal atypia and mitotic rate, the cases were classified as adenosarcomas. Sarcomatous overgrowth, presence of heterologous elements and high mitotic rate seem to indicate poor prognosis. Flow cytometry showed a predominantly diploid population of cells in eight cases of adenosarcoma. An aneuploid cell population was seen in three cases of adenosarcoma and in all but one case of carcinosarcoma studied as controls. Gynecologists and pathologists should be aware of the difficulties and delay in the diagnosis of Mullerian adenosarcoma in cases when the tumor presents as a benign looking cervical polyp. Flow cytometric DNA analysis might serve as a useful tool in the diagnosis of Mullerian adenosarcoma vs carcinosarcoma and contribute to the prognostic evaluation in different histologic subsets of the tumor.