Sclerotic Stroma Research Articles

Perineuriomatous Melanocytic Nevi: A Case Series of Four Cases.

Melanocytic tumors with perineuriomatous differentiation may pose diagnostic challenges. This study explores characteristics of perineuriomatous melanocytic nevi, an entity merging features of perineurioma and melanocytic nevi. The aim is to elucidate histopathological features of perineuriomatous nevi to allow dermatopathologists to recognize them and differentiate them from other spindle cell lesions. This study reviews four cases (2020-2023) of melanocytic nevi with perineuriomatous components. All cases comprised adults (median age: 64.50, mean age: 60.25), with a female predominance, and exhibited clinical features characterized by irregular brown macules or papules on the trunk and extremities. Histopathologically, all lesions were compound, and biphasic patterns were evident, encompassing superficial nevoid and deeper spindled populations arranged in whorled fascicles and embedded in a sclerotic or myxoid stroma. Immunohistochemistry revealed expression of at least one perineuriomatous marker in deeper cells. Cases were assessed and compared with previously published cases for comprehensive insights. Three of our four cases demonstrated the presence of a junctional component that was smaller than the dermal component. We suggest that this unusual feature, which we term the "reverse shoulder", may allow dermatopathologists to help consider perineurial differentiation in the appropriate setting. Perineuriomatous nevi can pose diagnostic challenges. This study contributes to the growing body of literature on perineuriomatous nevi, emphasizing their unique features and the importance of accurate diagnosis to avoid unnecessary interventions.

Sclerosing Extramedullary Hematopoietic Tumor of the Breast

Abstract Introduction/Objective Sclerosing extramedullary hematopoietic tumor (SEMHT) is a rare tumor that occurs in patients with chronic myeloproliferative conditions. SEMHT can be seen in abdominal organs, retroperitoneum and mesentery. It can also be regarded as an advanced manifestation of myelofibrosis. SEMHT can occur singly or as multiple masses and manifests later in the disease course of CMPD. The differential diagnosis includes breast cancer, soft tissue tumor, and extramedullary hematopoiesis. Here we present a case of SEMHT in the breast. Methods/Case Report This is a 80-year-old female, who has a history of left breast ductal carcinoma in situ who underwent lumpectomy and received radiation in 2020. The patient also has Polycythemia Vera and MF. Bone marrow aspiration, Trephine biopsy imprints and Trephine and clot biopsies showed persistent myelofibrotic myeloid neoplasm involving a cellular bone marrow with marked fibrosis (MF Grade 3), megakaryoblastic hyperplasia, atypical megakaryocytes. In 3/2023, there were two small masses found in upper outer right breast by ultrasound measuring 4 mm and 3 mm in diameter. Breast biopsy sections showed scattered rare atypical cells within fibrotic stroma. They were only positive for Vimentin and Androgen Receptor (rare) within the performed immunohostochemistry panel. Ki67 proliferation index was 20%. The biopsy report was signed out as aytpical mesenchymal tumor. Resection was recommended. The resection showed same atypical cells within large sclerotic stroma. These atypical cells were scattered in the breast tissue along with sinusoidal capillarization. The atypical cells were atypical megakaryocytes highlighted with CD61. Erythrocyte precursors are highlighted with glycophorin a. Based on the clinical history and histologic features, SEMHT diagnosis was made. Results (if a Case Study enter NA) NA due to being a case study. Conclusion SEMHT is a rare tumor, that can occur in patients with CMPD. It can occur within different locations in the body, including breast. SEHMT should be kept in mind for an accurate diagnosis of the tumor and correct management of the patients.

Sclerosing epithelioid fibrosarcoma: a new mesenchymal non-meningothelial tumor involving the central nervous system?

Sclerosing epithelioid fibrosarcoma (SEF) is a rare mesenchymal neoplasm defined in the WHO classification of soft tissue tumor as a "rare malignant fibroblastic neoplasm characterized by epithelioid fibroblasts arranged in cords and nests and embedded in a dense sclerotic hyalinized stroma." These morphological features are associated with a unique immunohistochemical pattern (particularly MUC4 hyperexpression) and with characteristic gene rearrangements, mostly involving EWSR1 and CREB3L1 or CREB3L2. If it usually arises in deep soft tissues of various locations, SEF has also been reported in bones or viscera. Nevertheless, to our knowledge, no primary brain location has been described. Here, we report a left occipital SEF arising in a middle-aged woman with no evidence of systemic location after a comprehensive clinic-radiological assessment. We discuss the main differential neuropathological diagnoses and provide a comprehensive genetic and epigenetic description of this tumor comparatively to the more frequent systemic locations.

Abstract NG02: Distinct genomic and immunologic tumor evolution in germline TP53-driven breast cancers

Abstract NG02: Distinct genomic and immunologic tumor evolution in germline TP53-driven breast cancers

Intra-Abdominal Epithelioid Neoplasm With EWSR1::CREB Fusions Involving the Kidney: A Clinicopathologic and Molecular Characterization With an Emphasis on Differential Diagnosis

Soft tissue neoplasms, harboring fusions between EWSR1 and FUS with genes encoding CREB transcription factors family (ATF1, CREB1, and CREM), are an emerging heterogeneous group of mesenchymal tumors that differ significantly in morphology, immunophenotypes, and behavior. Recently, EWSR1/FUS::CREB fusions have been recognized to define a group of aggressive neoplasms of epithelioid morphology with multiple growth patterns and a striking predilection for mesothelial-lined cavities. These neoplasms presenting as a primary neoplasm of intra-abdominal visceral organs are rare, which could elicit a wide range of differential diagnoses because of their diverse morphologies and immunohistochemical profiles. We report 3 cases of intra-abdominal epithelioid neoplasms with EWSR1::CREB fusions involving the kidney. This study included 2 female patients and 1 male patient, with age at presentation ranging from 17 to 61 years (mean: 32 years). All the patients underwent radical nephrectomy without adjunctive therapies. Grossly, the tumors were large, and all were solitary masses with sizes ranging from 5.6 to 30.0 cm (mean: 14.5 cm). Histologically, the neoplasms showed infiltrating and indistinct borders and were composed predominantly of monomorphic round-to-epithelioid cells with variable amounts of pale-to-clear cytoplasm, arranged in cords, nests, and sheets and embedded in a sclerotic hyalinized stroma with variable lymphoid cuffing either intermixed or at the periphery. Notably, a hemangiopericytomatous growth pattern was commonly seen. Nuclear atypia was mild, and mitotic activity was scarce. Immunohistochemically, all 3 cases were at least focally positive for epithelial membrane antigen and keratin AE1/AE3, with 2 tumors showing focal MUC4 expression and 1 case displaying diffuse CD34 and focal CAIX positivity. Targeted RNA sequencing identified EWSR1::CREM fusion in 2 cases and EWSR1::ATF1 fusion in 1 case. Subsequent fluorescence in situ hybridization analysis confirmed the RNA sequencing results. On follow-up, 1 patient developed multiple spinal bone metastases 5 months after the surgery while the other 2 patients were free of disease 9 and 120 months after diagnosis, respectively. Our findings demonstrate that intra-abdominal epithelioid neoplasms with EWSR1::CREB fusions may rarely occur primarily in the kidney and should be included in the differential diagnosis of primary renal epithelioid mesenchymal neoplasms.

Funnel and Vessel-Duct Adenoma Breast

Duct adenoma appears as an exceptionally discerned, benign tumefaction comprised of distorted glandular articulations circumscribed within a fibrous tissue capsule and intermingled within a sclerotic stroma. Initially scripted Azzopardi and Salm in 1984, the adenomatous lesion is configured of miniature and intermediate ducts disseminated within peripheral breast tissue. Alternative nomenclature of sclerosing papilloma is not recommended.

Sclerosing Perivascular epithelioid cell neoplasms (PEComa), a rare variant in a unique family of tumors

Abstract Introduction/Objective Perivascular epithelioid cell neoplasms (PEComas) are a rare group of neoplasms that characteristically show both smooth muscle and melanocytic differentiation. The most common tumor in this family is renal angiomyolipoma but may occur in various sites. Sclerosing PEComa (S-PEComa) is an extremely rare variant. its only reported in females and it is usually an incidental finding in the retroperitoneum, in close proximity to the kidney. Methods/Case Report . We present a 52-year-old female, who had a chest CT scan for worsening cough. An - 4 cm exophytic renal mass in the left renal upper pole was found. Patient underwent left partial nephrectomy. Grossly the specimen consisted of 2.5 cm well-circumscribed, white tan mass, abutting the renal capsule, and extending into perinephric adipose tissue. Histology revealed uniform bland epithelioid cells with palely eosinophilic cytoplasm and round nuclei embedded in abundant densely sclerotic stroma. No fat was identified. Immunohistochemistry showed the tumor to be positive for desmin, HMB-45 and SMA with focal staining for AE1/AE3 and negative staining for EMA, cam 5.2, CD 117, calretinin, CD 34, S-100, PAX 8, and Melan A, which confirming the diagnosis of S-PEComa. Results (if a Case Study enter NA) NA Conclusion PEComas, excluding the epithelioid variant, usually has a benign course. In the 13 cases previously reported S-PEComa, 2 patients had metastasis. Our patient, 3-month post-op, has no evidence of disease, but close follow up still warranted.

Biphasic synovial sarcoma with myoepithelial features: a distinctive variant with a predilection for the foot.

Synovial sarcoma (SS) is a tumor known for its classic monophasic spindle cell or biphasic morphology. However, it exhibits a wide range of histologic variations, leading to diagnostic challenges. Here, we present four cases of molecularly confirmed, biphasic SS originating in the feet and displaying myoepithelial differentiation. The patients were two men and two women with an age range from 19 to 71 years (mean, 45 years). Each tumor showed foci with conventional spindle cell morphology. The epithelial components included areas with nests and cords of epithelioid cells set within a hyalinized and sclerotic stroma. The cytoplasm was clear to pale and eosinophilic. The nuclei were ovoid-round with fine chromatin and small to inconspicuous nucleoli. Mitotic figures were present (2-13 per 10 high-power fields; mean, 6.5). Immunohistochemical studies showed variable staining of the myoepithelial-like regions for low molecular weight keratins, EMA, p63, and S100 protein. Molecular studies confirmed the presence of SS18::SSX1/2 fusion in all four tumors. These cases highlight an unusual variant of synovial sarcoma with an apparent predilection for the distal lower extremity and suggest that differentiation of biphasic synovial sarcoma may be impacted by the anatomic site. Awareness of this variant is important to avoid misclassification and potential treatment and prognostic implications.

Imaging and Management of Radial Scars and Complex Sclerosing Lesions.

Radial scars and complex sclerosing lesions, often collectively referred to as radial sclerosing lesions (RSLs), are breast lesions characterized by sclerotic stroma with entrapped epithelial elements. RSLs have imaging features that overlap with those of breast malignancy and often become the target of imaging-guided biopsy given their suspicious imaging appearance. These can be identified in isolation or can also be associated with atypia or other high-risk lesions that have intrinsic malignant potential, increasing the risk of carcinoma and affecting prognosis and management of RSLs. Because of this, management of these lesions remains controversial. Traditional management has been surgical excisional biopsy. However, as more RSLs are identified (because digital breast tomosynthesis allows identification of more architectural distortions), optimal management is evolving. Physicians in some practices are using a multidisciplinary approach to the management of RSLs when deciding on surgical excision of these lesions versus imaging follow-up. These discussions also incorporate individual patient risk factors and greater patient informed medical decision making. Reported upgrade rates of RSLs at core needle biopsy vary and can depend on the sampling method, number of samples, gauge of the needle, target being sampled, and radiologic-pathologic concordance or discordance. A precise sampling technique also allows greater accuracy of diagnosis and lower upgrade rates for these lesions, with radiologic-pathologic correlation as an integral component for further management decisions. The authors review the overall histopathologic, clinical, and imaging features of RSLs and discuss appropriate management based on currently available data regarding upgrade rates. ©RSNA, 2023 Quiz questions for this article are available through the Online Learning Center.

GRM1 Gene Fusions as an Alternative Molecular Driver in Blue Nevi and Related Melanomas

Activating mutations in GNAQ, GNA11, CYSLTR2, and PLCB4 genes are regarded as the main oncogenic drivers of blue nevi (BN) and blue malignant melanocytic tumors. Here we report 4 cases of blue melanocytic neoplasms devoid of these mutations but harboring GRM1 gene fusions. In this short series, there was no gender predominance (sex ratio, 1). The mean age at diagnosis was 40 years (range, 12-72). Tumors were located on the face (n = 2), forearm (n = 1), and dorsum of the foot (n = 1). Clinically, a plaque-like pre-existing BN was found in 2 cases, including a deep location; another case presented as an Ota nevus. Two cases were diagnosed as melanoma ex-BN, one as an atypical BN, and one as a plaque-like BN. Microscopic examination revealed a dermal proliferation of dendritic melanocytes in a sclerotic stroma. A dermal cellular nodule with atypia and mitotic activity was observed in 3 cases. Genetic investigation by whole exome RNA sequencing revealed MYO10::GRM1 (n = 2) and ZEB2::GRM1 (n = 1) fusions. A GRM1 rearrangement was identified by fluorescence in situ hybridization in the remaining case. SF3B1 comutations were present in the 2 melanomas, and both had a MYO10::GRM1 fusion. Array comparative genomic hybridization was feasible for 3 cases and displayed multiple copy number alterations in the 2 melanomas and limited copy number alterations in the atypical BN, all genomic profiles compatible with those of classical blue lesions. GRM1 was overexpressed in all cases compared with a control group of blue lesions with other typical mutations. Both melanomas rapidly developed visceral metastases following diagnosis, with a fatal outcome in one case and tumor progression under palliative care in the other. These data suggest that GRM1 gene fusions could represent an additional rare oncogenic driver in the setting of BN, mutually exclusive of classical canonical mutations, especially in plaque-type or Ota subtypes.

Acral BRAF‐mutated tubular adenoma should be distinguished from HPV42‐related digital papillary adenocarcinoma

Acral <i>BRAF</i>‐mutated tubular adenoma should be distinguished from <scp>HPV42</scp>‐related digital papillary adenocarcinoma

Multidisciplinary management in the treatment of intrahepatic cholangiocarcinoma.

Multidisciplinary management in the treatment of intrahepatic cholangiocarcinoma.

Fibroma of tendon sheath: a clinicopathological and genetic analysis of 134 cases

Objective: To investigate the clinicopathological features, immunophenotypes and molecular genetics of fibroma of tendon sheath (FTS). Methods: One hundred and thirty-four cases of FTS or tenosynovial fibroma diagnosed in the Department of Pathology, West China Hospital, Sichuan University, Chengdu, China from January 2008 to April 2019 were selected. The clinical and histologic features of these cases were retrospectively reviewed. Immunohistochemistry, fluorescence in situ hybridization (FISH) and reverse transcription-polymerase chain reaction (RT-PCR) were performed on the above cases. Results: There were a total of 134 cases of FTS, including 67 males and 67 females. The patients' median age was 38 years (ranged from 2 to 85 years). The median tumor size was 1.8 cm (ranged from 0.1 to 6.8 cm). The most common site was the upper extremity (76/134, 57%). Follow-up data was available in 28 cases and there was no detectable recurrence. Classic FTS (114 cases) were well-defined and hypocellular. A few spindle-shaped fibroblasts were scattered in the dense collagenous sclerotic stroma. Characteristically elongated slit-like spaces or thin-walled vessels were observed. Most of cellular FTSs (20 cases) were well-defined and the area with increased cellularity of the spindle cells coexisted with classic FTS. There were occasional mitotic figures, but no atypical mitotic figures. Immunohistochemistry was performed in 8 cases of classic FTS and most cases were positive for SMA (5/8). Immunohistochemistry was also performed in 13 cases of cellular FTS and showed 100% positive rate for SMA. FISH was conducted on 20 cases of cellular FTS and 32 cases of classical FTS. USP6 gene rearrangement was found in 11/20 of cellular FTS. Among 12 cases of CFTS with nodular fasciitis (NF)-like morphological feature, 7 cases showed USP6 gene rearrangement. The rearrangement proportion of USP6 gene in cellular FTS without NF-like morphological features was 4/8. By contrast, 3% (1/32) of the classic FTS showed USP6 gene rearrangement. RT-PCR was performed in those cases with detected USP6 gene rearrangement and sufficient tissue samples for RT-PCR. The MYH9-USP6 fusion gene was detected in 1 case (1/8) of the cellular FTSs, while no target fusion partner was detected in the classic FTS. Conclusions: FTS is a relatively rare benign fibroblastic or myofibroblastic tumor. Our study and recent literature find that some of the classic FTS also show USP6 gene rearrangements, suggesting that classical FTS and cellular FTS are likely to be at different stages of the same disease (spectrum). FISH for USP6 gene rearrangement may be used as an important auxiliary diagnostic tool in distinguishing FTS from other tumors.

Sclerosing Central Mucoepidermoid Carcinoma: Rare Case Series and Review

Introduction:&#x0D; The most frequent malignant tumour of the salivary glands is mucoepidermoid carcinoma which accounts for 30% of all malignancies of the salivary glands. There are numerous histologic variations with various levels of differentiation. Sclerosing MEC (SMEC) is a rare subtype of MEC that has the potential to be misdiagnosed as a low-grade non-SMEC malignancy or a benign reactive condition. There have been no cases of the extremely rare sclerosing variant of central or intraosseous MEC reported as of yet.&#x0D; Case reports:&#x0D; We present two cases of Sclerosing Central MEC, in which histologic analysis revealed relatively well-circumscribed, nonencapsulated tumours made up of dispersed epithelial islands of low-grade MEC and extensive central sclerosis. Similar sclerotic stroma was present in the tumor in the second case, but the epithelial component was of intermediate grade. Both cases showed abundant intracytoplasmic mucin and positive Mayer mucicarmine and PAS stains.&#x0D; Conclusions:&#x0D; Sclerosing central mucoepidermoid carcinoma was identified as the cause. Both cases underwent a complete resection of the tumor and have since been free of the disease.

Wide morphological spectrums of renal neoplasms with TSC/mTOR, NF2, or monoallelic MUTYH germline mutation

Advances in molecular biology techniques have led to the recognition of more molecularly defined renal entities. However, some molecular-defined renal cell carcinomas (RCC) exhibit heterogeneous morphologies. In recent years, TSC/mTOR and NF2 alterations in renal cancers have attracted considerable attention from pathologists, including several renal tumors that have been validated as different entities. The monoallelic MUTYH germline mutation has also recently been demonstrated to be a driver of tumorigenesis, which is relatively enriched in renal cancers and may predict the risk of metastasis. In the current study, we presented seven cases with TSC/mTOR alterations (two with fibromyomatous stroma, one concomitant with ELOC mutation, three eosinophilic solid and cystic RCC, and two RCC, NOS), four cases with NF2 mutations (one concomitant with FH mutation, one biphasic hyalinizing psammomatous RCC, and two RCC, NOS), and two cases with monoallelic MUTYH germline mutations (one collecting duct carcinoma and one papillary RCC). We observed that renal cancers harboring these mutations had a wide morphological spectrum. Renal neoplasms with TSC/mTOR mutations may have an indolent outcome. NF2-mutated RCC appeared to display calcification and sclerotic stroma. The identification of these renal cancers can help reduce the number of tumors diagnosed as RCC, NOS. However, whether they can be grouped into TSC/mTOR, NF2, or MUTYH -associated RCC requires further validation.

Chronicles of A.I. Pospelov Moscow Society of Dermatovenereologists and Cosmetologists (MSDC was founded on October 4, 1891). Bulletin of the MSDC № 1149

On October 18, 2022, the 1149th meeting of the A.I. Pospelov Moscow Society of Dermatovenerologists and Cosmetologists (MSDC) took place. The meeting was held in a face-to-face format. There were 128 participants in total. Accepted as a member of the MSDC 53 people.&#x0D; Two clinical cases are presented for discussion: trichoadenoma ― a rare benign follicular tumor consisting of strands of epithelial cells and keratinizing cysts embedded in the sclerotic stroma, and Dariers follicular dyskeratosis ― hereditary dermatosis characterized by the formation of follicular hyperkeratotic vegetative papules on seborrheic areas of the skin caused by a violation of the keratinization process by the type of dyskeratosis.&#x0D; Scientific reports were devoted to the analysis of differential diagnosis and therapeutic tactics in BrookeSpiegler syndrome (multiple cylindromes and trichoepitheliomas), the mechanisms of action and effectiveness of zinc pyrithione as a means of controlling colonization of the hairy skin by fungi-commensals of the genus Malassezia, as well as the most urgent problems of modern medicine ― the features of the course of chronic-severe dermatoses after SARS-CoV-2 infection (COVID-19) and an increase in the incidence of mucormycosis, which is a marker of immune breakdown that occurs immediately after COVID-19 infections.

A case of sclerosing odontogenic carcinoma of the mandible with a review of the literature.

Sclerosing odontogenic carcinoma (SOC) is a very rare malignant odontogenic tumor characterized by sclerotic stroma and single-file cord-like tumor cell structures. A 38-year-old man presented with extraoral swelling and right mental region paralysis. Panoramic radiography revealed an ill-defined radiolucent lesion extending from the right mandibular ramus to the right lower canine. Magnetic resonance imaging showed tumor invasion into the right inferior alveolar nerve and masseter muscle. Hemimandibulectomy, bilateral neck dissection, and mandibular reconstruction were performed using a rectus abdominis musculocutaneous flap and a titanium plate. Histopathology and immunohistochemistry confirmed SOC diagnosis. No recurrence occurred in the 1-year follow-up. In this paper, a case of SOC with a high Ki-67 labeling index was reported. Since SOC is prone to nerve invasion, treatment is resection with an appropriate surgical margin.

Follicular Lymphoma Mimicking ‘Adenocarcinoma with Clear Cells and Signet Ring Morphology’

Case Presentation 91-year-old female who presented with epigastric pain. CT showed a 13 cm mass in the right lower quadrant encasing the right colon and enlarged lymph nodes. Histology of the CT guided biopsy of the mass showed malignant tumour which was composed mainly of cells with clear, vacuolated cytoplasm and eccentric nuclei (signet-ring like). The cells were surrounded by sclerotic stroma and arranged in a nested configuration (Image 1). Morphological features of carcinoma including keratinisation and glandular differentiation were not evident. Melanin pigment in indicate melanoma was not seen. On immunohistochemistry broad spectrum cytokeratin (AE1/3 and CAM5.2) and SOX10 were negative which would exclude carcinoma and melanoma respectively. The tumour was diffusely positive with CD45 and CD20 confirming B-cell lineage (Image 2). There was positivity with BCL2 and germinal centre makers BCL6 and CD10. Staining with antibodies to CD21 and CD23 confirms the presence of at least partial follicular architecture.

The clinicopathological spectrum of sclerosing epithelioid fibrosarcoma: report of an additional series with review of the literature

We present a case series of sclerosing epithelioid fibrosarcoma (SEF) to further characterise its clinical and pathological features. Twenty-one patients with SEF were included in this study. There were 12 males and nine females (range 25-63 years; median 38 years). Tumours were located in the kidney (n=5), thigh (n=3), chest wall (n=3), head and neck (n=2), bone (n=2), abdominal wall (n=1), psoas major (n=1), retroperitoneum (n=1), omentum (n=1), popliteal space (n=1) and lung (n=1). Tumour sizes ranged from 2.5 to 16 cm (median 7 cm). Microscopically, epithelioid tumour cells were arranged in nests and cords and embedded in a dense sclerotic stroma. Some tumours showed myxoid areas, fibroma-like areas, acinar growth patterns and haemangiopericytoma-like appearance. A few tumour cells presented a rhabdomyoid shape. Calcification, ossification, cystic and necrosis were observed in some cases. The diagnosis was confirmed by immunoreactivity for MUC4, and by further fluorescence in situ hybridisation (FISH) or next generation sequencing (NGS) analysis. Clinical follow-up was available for 16 cases (median, 24 months; range 6-62 months). Seven patients developed metastases to lung (n=3), bone (n=3), brain (n=2) and back (n=1). Four patients developed a local recurrence. Three patients died of disease. Overall survival (OS) of SEF was related to patient age (p=0.001) and progression-free survival (PFS) was related to tumour size (p=0.046). In addition to soft tissue, SEF is more likely to involve the viscera and the abdominal cavity and has morphological variants. Familiarity with its distinctive clinical and pathological features helps avoid misdiagnosis.

Review of Sclerosing stromal tumor: A relatively rare ovarian neoplasm

Abstract Introduction/Objective Sclerosing stromal tumor (SST) is sex cord stromal tumor which is rare primary ovarian neoplasm. Because its clinical presentation and imaging findings accurate preoperative clinical diagnosis can be difficult. We report three cases of a sclerosing stromal tumor of ovary and histopathological presentation. Methods/Case Report Our patients had an age ranged from 27-40 years with primary ovarian neoplasm and as incidental in 64-year-old patient. Radiologically lobulated and heterogeneously enhancing, partially calcified masses with nodular rind of avid enhancement about the periphery of the lesion with high risk malignancy were seen. Grossly the masses had firm tan-white, solid, lobulated cut surfaces with patchy calcifications Results (if a Case Study enter NA) Microscopically all the three tumors showed a hyalinized, well-circumscribed nodule with few scattered trabeculae of spindled to plump cells with pale grey cytoplasm within a sclerotic stroma with few staghorn- like blood vessels around the periphery. Scattered calcifications were identified. No mitoses are identified. The reticulin stain showed pericellular focal nested staining pattern. There was no necrosis, pleomorphism, or features of malignancy were identified. Findings were consistent with a sclerosing stromal tumor. Conclusion Ovarian sex cord-stromal tumors are relatively infrequent neoplasms of all primary ovarian tumors. Clinical and sonographic findings alone do not predict the presence of this tumor. SSTs should be considered in differentials in young females with unilateral masses with cystic and solid masses. These tumors carry a good prognosis. Histopathological features along with immunohistochemsitry establish the diagnosis.