Benign Stromal Tumor Research Articles

Ovarian fibroma: A diagnostic dilemma

Introduction: Ovarian fibroma is a benign stromal tumor composed of spindle fibroblasts like cells that produce collagen. It is seen in perimenopausal and postmenopausal women and is seldom seen in women with the age of less than 30 years. Case description: A young lady presented with irregular menses and secondary amenorrhoea of 8 months with gradual distension of abdomen. Raised Ca-125 levels with CECT impression raised the suspicion of malignant right ovarian tumor. Staging laparotomy was done followed by right salpingo-ovariotomy. Histopathology with immunohistochemistry confirmed the diagnosis of ovarian fibroma. Conclusion: There are no specific markers for an accurate pre-operative diagnosis of an ovarian fibroma. Improved understanding of this entity is essential to prevent misdiagnosis and missing the diagnosis.

A Case Report of Breast Fibromatosis Mimicking Breast Cancer

Introduction: Breast Fibromatosis is a rare benign stromal tumor that accounts for 0.2% of all breast tumors. The clinical presentation and imaging features may mimic a malignant tumor. We reported a case of breast fibromatosis mimicking breast cancer.Case Presentation: A 22-years-old woman with a palpable breast lump was present and being examined. The ultrasound and mammography features suggested malignancy. Magnetic Resonance Imaging (MRI) and Multislice Computerized Tomography (MSCT) evaluation showed invasion of a tumor to muscle without intrathoracic involvement. A core biopsy was performed with the pathologic conclusion suspected as a phyllodes tumor with a differential diagnosis as mammary fibromatosis. The pathology result of intraoperative examination favor for fibromatosis. A wide excision procedure with a free margin could be achieved. The histopathological examination revealed spindle cells tumor infiltrating into muscle tissue. The result of immunohistochemistry examination excluded metaplastic carcinoma and Phyllodes tumor. Therefore, it confirmed a diagnosis of breast fibromatosis.Conclusions: Breast fibromatosis is a rare benign tumor of the breast that can mimic malignancy. This entity should be considered as one of the differential diagnoses in a patient with a breast lump. A pre-operative tissue diagnostic is mandatory to prevent radical treatment for this nonmalignant case.

A Rare Mesenchymal Neoplasm in an Uncommon Anatomical SiteSolitary Fibrous Tumour of Vulva

Previously, Solitary Fibrous Tumour (SFT) was thought to originate from the pleura, a relatively rare mesenchymal neoplasm with an indolent course. Vulva is the very uncommon site of extrathoracic SFT. This is the report of a very uncommon case of vulval SFT in a 30-year-old female with complaint of a swelling over vulval region. Fine Needle Aspiration Cytology (FNAC) of the growth was attempted twice and only blood was aspirated. The swelling was excised completely. On histological examination, the sections revealed a cellular tumour of ovoid-to-fusiform spindle cells with indistinct cell borders, with haphazard arrangement or patternless pattern along with proliferation of variable sized blood vessels. The diagnosis was finalised to be a benign stromal tumour (most probably SFT), which was confirmed on immunohistochemical examination using Cluster of Differentiation 34 (CD34), CD99 and Signal Transducer and Activator of Transcription 6 (STAT6) markers.

Diagnostic performance of multi-parametric MRI to differentiate benign sex cord stromal tumors from malignant (non-stromal and stromal) testicular neoplasms.

Testicular stromal tumors are uncommon, although mostly benign. The purpose of this study is to assess the role of multi-parametric MRI in differentiating benign testicular stromal tumors from malignant testicular neoplasms (non-stromal and stromal). A single-center retrospective study comparing benign stromal tumors (STs) to malignant testicular neoplasms (MTNs) was conducted. MR imaging assessment included tumor size, T2- and T1-weighted signal intensity, T2- and T1-weighted texture pattern, diffusion restriction, presence of hemorrhage and/or necrosis, and measurement of apparent diffusion coefficient and dynamic contrast enhancement (DCE). Inter-observer agreement was assessed using Cohen's kappa and Bland-Altman and data were compared using independent t-tests or χ2. Receiver operating characteristic curve analysis was used to test models incorporating various imaging features. Radical orchiectomy and histopathology revealed 20 testicular neoplasms: seven STs (35%) and thirteen MTNs (65%). MTNs were significantly larger in size than STs (5.1 ± 2.36cm vs. 1.27 ± 0.56cm; p-value < 0.001). STs demonstrated more hypointense T2W signal (85.7% vs. 46.2%; p-value < 0.09), less T2W heterogeneous texture (14.3% vs. 61.5%; p-value < 0.04), and less diffusion restriction (16.7% vs. 83.3%; p-value < 0.01) in comparison to MTNs. STs demonstrated mainly homogenous post-contrast enhancement pattern (71.4% vs. 7.7%; p-value < 0.004), while MTNs showed primarily heterogeneous enhancement pattern (77% vs. 14.3%; p-value < 0.02). STs revealed greater corrected venous phase enhancement (STs: 0.59 ± 0.29; MTNs: 0.25 ± 0.25; p-value < 0.03). STs showed higher ADC values, though the difference was not statistically significant (p-value < 0.25). A model combining T2W, DWI, and DCE features showed the best overall diagnostic accuracy with area under ROC curve of 0.99 and confidence interval ranging from 0.94 to 1. Multi-parametric MRI can potentially differentiate benign stromal tumors from malignant testicular neoplasms, which can help to avoid radical orchiectomy. However, future studies using larger sample sizes are needed to validate our results.

Male mammary myofibroblastoma: Two case reports and brief review of literature.

Myofibroblastoma of the breast is a rare benign stromal tumor that occurs in both sexes with a higher prevalence in male breast of older populations. Furthermore, myofibroblastoma can arise in extra mammary sites, along the milk-line. A variety of morphological variants in addition to the classic type have been identified. The differential diagnosis includes both benign and malignant entities that, through the use of clinical and radiological imaging, is difficult to characterize. Histopathological examination and immunohistochemistry are fundamental in the establishment of appropriate management of the disease and avoidance of overtreatment. The present study focuses on two cases of male mammary myofibroblastoma, with a short literature review.

Mammary myofibroblastoma: A case report of a rare benign tumour in an elderly man

Mammary myofibroblastoma is a rare benign stromal tumour, predominantly occurring in menopausal women and older men. We present a case of a 73-year-old man with a tender right breast lump. He underwent a core biopsy assessment followed by a wide local excision. The lesion was a well circumscribed spindle cell nodule with interlacing fascicles and intervening collagen. The cells are elongate with pale cytoplasm and regular nuclei. There are scattered cells showing mild pleomorphism. Mitoses are uncommon. Multiple immunohistochemical stains were performed which were the key to diagnosis of myofibroblastoma. Completely excised lesions are not known to recur.

Hemangioblastoma-like Clear Cell Stromal Tumor of the Lung: A Clinicopathologic and Immunohistochemical Study of 5 Cases.

Five cases of an unusual primary benign stromal tumor designated as hemangioblastoma-like clear cell stromal tumor of the lung are presented. The patients are 4 women and 1 man between the ages of 39 and 52 years of age (average: 45.5 y). The patients presented with nonspecific symptoms of cough, chest pain, or dyspnea. None of the patients had any prior history of malignancy or tumor elsewhere. Diagnostic imaging showed the presence of an intrapulmonary tumor. Lobectomy was performed in all 5 patients. Grossly, the tumors were well-demarcated but not encapsulated with focal areas of hemorrhage without necrosis. Histologically, low power examination showed a cellular proliferation alternating with discrete dilated vessels reminiscent of a vascular neoplasm. Higher magnification showed medium-sized cells with clear cytoplasm arranged in sheets and cords. Mitotic activity and marked cellular atypia were not present. A wide panel of immunohistochemical studies was performed including epithelial, neural, muscle, vascular, and neuroendocrine markers, all of which showed negative staining. Tumor cells showed positive staining for vimentin. In 2 cases, fluorescence in situ hybridization for the solitary fibrous tumor was performed and was negative. Clinical follow-up in 3 patients showed no evidence of recurrence. The cases herein presented highlight an unusual benign stromal tumor of the lung, which needs to be considered in the differential diagnosis of tumors with a clear cell and vascular appearance.

Benign ovarian lesions with restricted diffusion.

Developments in magnetic resonance imaging have expanded its role in the assessment of the female pelvis, including the diagnosis of ovarian lesions. In this setting, diffusion-weighted imaging has proven its diagnostic value, which is particularly important in differentiating between benign and malignant ovarian tumors. In general, the latter show restricted diffusion, whereas the former do not. Exceptions include teratomas, endometriomas, hemorrhagic cysts, ovarian abscesses, ovarian infarction, and some benign stromal tumors. The aim of this review is to draw attention to benign ovarian lesions with restricted diffusion, with a special focus on diffusion-weighted imaging pearls and pitfalls.

Renal Cystic Nephroma: Histologic and Immunohistochemical Analyses and Review of Literature

Background: A case of renal Cystic Nephroma (CN) was reported in 32 years-old women presenting with an incidental renal mass on radiological imaging. Methods: On CT-scan examination, a renal cell carcinoma was suspected and right nephrectomy was performed. The resected tumor measured 17 × 10 × 7 cm. Macroscopically, the tumor was a multilocular mass with cysts ranging from 0.5 cm to 4.5 cm. The tumor was well delineated relative to non-tumoral renal parenchyma, which was physiological in appearance with a cortex at 1 cm without evidence of infiltration of the tumor. At the hilum, the arterial, venous and ureter were normal. No lymph node was identified. Microscopically, the lesion was well defined by a fibrous capsule, consisting of multiple cystic formations of variable sizes bordered by a mono-stratified epithelium, which ranged from flat to cubic. The lining epithelial cells had a hobnail appearance, abundant cytoplasm and an oval nucleus associated with a nucleolus. No mitosis were founded. The cysts were subtended by cellular fibrous septa of ovarian type. On immunohistochemical examination, the epithelial cells were positive for cytokeratins 7 and 19; stromal cells were positive for estrogen receptors, progesterone receptor and CD10. In the septa, there were some bundles of smooth muscle cells positive for desmin and smooth muscle actin. Conclusion: The patient was alive 8 years after treatment, without metastasis or recurrence. Histologic and immunohistochemical analyses may be helpful in differential diagnosis between benign cystic renal neoplasms, CN, mixed epithelial and stromal tumor (MEST) and malignant tumors such as clear cell renal carcinoma (CCRC) in adults.

Surgical Resection of Retroperitoneal Aggressive Angiomyxoma: Case Report and Review of the Literature.

Aggressive angiomyxoma is a benign stromal tumor with a higher prevalence in middle-aged women. The objective of this case report is to illustrate the aggressive clinical behavior of this benign tumor. We present the case of a 45-year-old female patient, with tumor recurrence after multiple surgical resections of a pelvis-subperitoneal angiomyxoma. Surgical excision of the tumor, with extensive pelvic dissection and organ resection, was performed. The 12-month follow-up showed no tumor recurrence. Based on this case, and the published literature we may conclude that surgical resection represents the main treatment of aggressive angiomyxoma, even though it is associated with significant morbidity and a poor local control of the tumor.

Epithelioid Myofibroblastoma in an Old-Male Breast: A Case Report with MRI Findings.

Myofibroblastoma of the breast (MFB) is a very rare benign stromal tumor. In recent years, increase in mammographic screenings has resulted in increased diagnosis of MFB. Most cases are old males and postmenopausal women. MFB may be confused as malignant, clinically, morphologically, or by imaging. Immunohistochemistry is essential for final diagnosis in these cases. We report a case of a pathologically diagnosed MFB in an 80-year-old male patient who had coexisting prostate cancer and describe its imaging characteristics, especially magnetic resonance imaging (MRI). In this paper, histopathological and MRI findings of the MFB were discussed.

Mammary myofibroblastoma with extensive myxoedematous stromal changes: A potential diagnostic pitfall

Myofibroblastoma (MFB) of the breast is a relatively rare, benign stromal tumor arising in the breast of both males and females. Several morphological variants have been recognized in the last two decades, including infiltrating, cellular, fibrous/collagenized, epithelioid/deciduoid-like cell, lipomatous and myxoid variants. Myxoid MFB is an extremely rare variant which can represent a potential diagnostic pitfall, posing differential diagnostic problems with other myxoid lesions occurring primarily in the breast parenchyma. We first report an unusual case of mammary MFB showing so extensive myxoedematous stromal changes that obscured the underlying neoplastic cells, rendering difficult its recognition as MFB. Tumor was composed of a few bland-looking spindled, stellate cells embedded in an abundant myxoedematous stroma containing thin and thick collagen fibers and numerous blood vessels with fibrin and foamy histiocytes deposits in their walls. Identification of a small-sized cellular area, with morphological and immunohistochemical features consistent with mammary-type MFB, was crucial for a correct diagnostic interpretation of the lesion. These unusual stromal and vascular features are likely morphological changes to which undergo long-standing mammary MFBs in response to ischemic, traumatic or inflammatory stimuli. Differential diagnostic problems with benign and malignant myxoid lesions of the breast parenchyma are discussed. The present case, for which the term “mammary MFB with extensive myxoedematous stromal changes” is proposed, contributes to widen the morphological spectrum of this unusual mammary tumor, suggesting that when pathologist is dealing with a hypocellular myxoedematous lesion of the breast, a MFB should be ruled out.

Spectrum of laparoscopic surgery for gastric tumors

Minimally invasive operative procedures are increasingly being used for treating tumors of the upper gastrointestinal tract. While minimally invasive surgery (MIS) has become established as a standard procedure for benign tumors and gastrointestinal stromal tumors (GIST) based on current studies, the significance of MIS in the field of gastric cancer is the topic of heated debate. Until now the majority of studies and meta-analyses on gastric cancer have come from Asia and these indicate the advantages of MIS in terms of intraoperative blood loss, minor surgical complications and swifter convalescence although without any benefits in terms of long-term oncological results and quality of life. Unlike in Germany, gastric cancer in Asia with its unchanged high incidence rate, 50 % frequency of early carcinoma and predominantly distal tumor localization is treated at high-volume centres. Due to the proven marginal advantages of MIS over open resection described in the published studies no general recommendation for laparoscopic surgery of gastric cancer can currently be given.

Juvenile Granulosa Cell Tumor of the Testis: A Bilateral and Synchronous Case. Should Testis-sparing Surgery Be Mandatory?

Granulosa cell tumor of the testis is an infrequent stromal cell tumor that can be distinguished into adult and juvenile, the latter being more common. Juvenile granulosa cell tumor of the testis is a rare pathologic finding, accounting for 1.2%-3.9% of prepubertal testicular tumors. It is considered as a benign stromal sex cord tumor and is usually unilateral. Although radical surgery was previously considered the treatment of choice, testis-sparing surgery is now recommended in all cases where applicable. We report a bilateral synchronous juvenile granulosa cell tumor in a 6-month-old child treated with testis-sparing surgery and provide a review of the literature.

Synchronous gastric gastrointestinal stromal tumor and colon adenocarcinoma: a case report.

Gastrointestinal stromal tumors (GISTs) represent the majority of primary mesenchymal tumors of the gastrointestinal tract. They are generally considered to be solitary tumors and therefore the synchronous occurrence with other primary malignancies of gastrointestinal track is considered a rare event. Here we present the case of a 75-year-old man admitted to our hospital with a 10-day history of gastrointestinal bleeding. Colonoscopy revealed an ulcerative mass of 4 cm in diameter in the ascending colon. Gastroscopy revealed a bulge in the gastric body measuring 1 cm in diameter with normal overlying mucosa. Surgical intervention was suggested and ileohemicolectomy with regional lymph node resection along with gastric wedge resection was performed. Pathologic examination of the ascending colon mass showed an invasive moderately differentiated adenocarcinoma stage III B (T3N1M0). Grossly resected wedge of stomach showed a well circumscribed intramural tumor which microscopically was consistent with essentially benign gastrointestinal stromal tumor (according to Miettinen criteria). The patient did not receive additional treatment. Two years later the patient showed no evidence of recurrence or metastasis.

Mixed epithelial and stromal tumour of kidney

Benign mixed epithelial and stromal tumour of the kidney (MES-TK) is a recently described rare neoplasm. It shows a predilection for perimenopausal women. About 50 cases have been described in the English literature. The pathogenesis of MESTK is uncertain with deranged hormonal environment, including unopposed oestrogen implicated in the induction of periductal foetal mesenchy-mal cell proliferation. A translocation involving t(1;19)(p22;p13.1) was identified in one case. Recent evidence supports the theory of a single cell of origin with the capacity for epithelial and stromal differentiation. Though most MESTK are benign, a few cases with aggressive behaviour and malignant transformation have been reported. We describe another two cases of MESTK occurring in two female patients. The mode of presentation, operative procedures and pathology is described.

Case of rectal angioleiomyoma in a female patient

Angioleiomyoma represents a benign stromal tumor, which usually occurs in the subcutaneous tissue of the extremities, although its occurrence in the gastrointestinal tract is very rare. A case of rectal angioleiomyoma in a 40 year-old female patient is described here. Six months earlier, the patient suffered from periodical prolapse of an oval tumor from the anus, along with difficulties in bowel movement. A transanal extirpation of the tumor was performed. This is the first reported case in the English literature of a patient presenting with prolapsed angioleiomyoma of the rectum. During the immediate postoperative period, as well as 6 mo later, the patient had an unremarkable postoperative recovery.

Mammary and vaginal myofibroblastomas are genetically related lesions: fluorescence in situ hybridization analysis shows deletion of 13q14 region

Partial monosomy 13q, a chromosomal alteration originally reported in spindle cell lipoma, has also been documented in a few cases of mammary myofibroblastoma. Subsequently, a monoallelic loss of RB1 and FOXO1, located on 13q14, was identified in some cases of cellular angiofibroma, a benign stromal tumor of the lower female genital tract. This cytogenetic finding and the overlapping morphologic and immunohistochemical features shared by spindle cell lipoma, mammary myofibroblastoma, and cellular angiofibroma strongly suggest a histogenetic link among these tumors. Recently, we have emphasized morphologic and immunohistochemical similarities between mammary and vulvovaginal myofibroblastoma. The aim of the present study was to asses if these 2 tumors share the same chromosomal alteration. We studied the chromosome 13q14 region by fluorescence in situ hybridization analysis in a series of mammary and vaginal myofibroblastomas, with a readable signal in 7 of 13 mammary myofibroblastomas and 5 of 7 cases of vaginal myofibroblastomas. Despite histologic variation, most of the mammary (5/7) and vaginal (3/5) myofibroblastomas showed monoallelic deletion of FOXO1 in more than 22% of the cell populations. Our findings confirm that mammary myofibroblastoma is a tumor that exhibits chromosome abnormalities associated with the loss of the 13q14 region. In addition, we show for the first time that myofibroblastoma of the lower female genital tract also exhibits the same chromosomal abnormality, supporting the hypothesis that both tumors are in the spectrum of a single entity, likely arising from a common precursor cell.

Neonatal testicular tumour presenting as an acute scrotum

Juvenile granulosa cell tumour (JGCT) is a rare benign stromal cell tumour of the testis accounting for approximately 1% of all paediatric testicular tumours. Presenting primarily as a painless testicular mass, the tumour may be associated with undescended testis, hydrocele or testicular torsion. Abnormal karyotype has also been described. We describe an unusual case of a neonatal juvenile granulosa cell tumour presenting as an acutely swollen, tender testis, originally diagnosed as an acute hydrocele. We describe the management and review the literature pertaining to this rare differential diagnosis of a neonatal acute scrotum. Keywords: acute scrotum, granulosa cell tumour, neonatal, testicular tumour

Review of Myofibroblastoma of Breast and Its Most Common Mimickers

Myofibroblastoma (MFB) is characterized as a benign stromal neoplasm composed of uniform, bland-looking spindle cells that are often arranged in fascicles separated by thick band of collagenous stroma. Variable cellularity is common. Immunohistochemically, the spindle cells are positive for CD34, vimentin, BCL-2, ER, PR, focally positive for smooth muscle actin and negative for cytokeratin, S-100 and CD117. Although classic MFB is typically a bland-looking spindle cell tumor, some unusual morphologic variants may show worrisome malignant-looking cells. Recognition of MFB variants and its wide variety of mimickers is very important for pathologists to arrive at the correct diagnosis, and avoid misdiagnosis of malignancy.