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At least 19 recordsLinked to original sources
PubMed2026

Loss of Methylthioadenosine Phosphorylase (MTAP) Expression: A Potentially Useful Tool for Distinguishing Sarcomatoid Urothelial Carcinoma From Inflammatory Myofibroblastic Tumor.

Inflammatory myofibroblastic tumor (IMT) and sarcomatoid urothelial carcinoma (SarUC) can have striking histologic overlap but have significantly different prognoses and clinical management paradigms. Loss of methylthioadenosine phosphorylase (MTAP) protein expression by immunohistochemistry (IHC) serves as a useful surrogate for homozygous 9p21 deletion, a recurrent genomic alteration in urothelial carcinoma (UC). We analyzed MTAP expression by IHC in 65 SarUCs and 27 urinary tract IMTs to evaluate its utility in navigating this challenging differential diagnosis. Overall, MTAP loss was significantly more frequent in SarUC (55%) compared with IMT (4%) (P < .0001). Among 46 biphasic SarUCs with independently evaluable epithelial and mesenchymal components, divergent expression patterns were frequent. The most common pattern was retention of MTAP staining in both epithelial and mesenchymal components (19/46; 41% of cases), followed by selective retention of MTAP in the epithelial component and loss in the mesenchymal component (16/46; 35% of cases). MTAP loss was observed in both the epithelial and mesenchymal components in 11 out of 46 (24%) SarUC cases. None of the 46 biphasic SarUC cases showed selective MTAP loss in the epithelial component but retention in the mesenchymal component. MTAP IHC was also particularly valuable in assessing clonal relationships in 2 challenging biphasic cases in which the differential diagnosis included a collision between a noninvasive low-grade papillary UC and an IMT versus a subtle IMT-like SarUC arising in association with an overlying noninvasive low-grade papillary UC. Next-generation sequencing on a subset of cases (n = 11) was useful for confirming 9p deletion in cases with MTAP loss by IHC, and for demonstrating molecular hallmarks of urothelial neoplasia thereby providing additional diagnostic support for morphologically challenging SarUC cases with IMT-like morphology. Therefore, MTAP IHC can be useful in evaluating spindle cell lesions of the urinary tract, as loss is significantly more common in SarUC than in IMT, and enriched in the mesenchymal component of biphasic SarUC. However, MTAP loss can be seen in both entities, and the diagnosis of IMT-like spindle cell tumors in the urinary tract requires careful integration of morphologic, immunohistochemical, and molecular data.

Humans↗
PubMed1976

The clinical aspects of mesothelioma.

Three hundred and twenty-seven cases of mesothelioma accepted by a panel of pathologists have been used to construct a clinical picture of the disease. The cases analysed died between 1 January 1960 and 31 December 1969 and consisted of 267 pleural, 37 peritoneal, and 23 cases which could have arisen in either site. Two hundred and sixty-eight were in men and 59 in women and the disease appeared to be the same in women as in men. The mean age at death was 59-37 (+/- SD 9-89) years but ranged from 29 to 88 years. The mode of onset was insidious in all but a few cases and the mean interval before reaching the hospital was 3-39 (+/- SD 4-64) months for pleural and 3-08 (+/- SD 3-22) for peritoneal cases. Patients usually noticed a dull non pleuritic pain first but suffered some breathlessness, lassitude and weight loss by the time they reached hospital. On examination there was little evidence of disease apart from the signs of pleural effusion or thickening or ascites. Clubbing and signs of asbestosis were rare except in the peritoneal cases who more frequently gave an occupational history of heavy exposure and showed the radiological consequences of this. At the time when these patients were investigated diagnostic procedures were unrewarding and many patients were only diagnosed in retrospect. The prognosis was somewhat better for patients shown to have mainly epithelial cell tumours, 17-89 (+/- SD 18-26) months, predominantly spindle cell lesions surviving on the average only 7-98 (+/- SD 8-55) months and mixed tumours 11-3 months. The criteria for the early clinical diagnosis are described with a view of facilitating the search for effective treatment.

Adult↗
PubMed1977

So-called pseudosarcoma of the esophagus: nodal metastases of the spindle cell element.

A polypoid lesion of the esophagus with all the morphological features of a so-called pseudosarcoma produced nodal metastases of the spindle cell element. Ultrastructurally, these spindle cells showed only fibroblastic features. The findings in this case and a review of cases reported as pseudosarcoma of the esophagus lend little support for the contention that they differ from cases categorized as carcinosarcoma of the esophagus.

Aged↗
PubMed2026

Fibromatosis-Like Metaplastic Triple-Negative Breast Cancer: A Case Report.

Fibromatosis-like metaplastic carcinoma (FLMC) is an extremely rare subtype of metaplastic breast carcinoma that closely resembles desmoid-type fibromatosis histologically, making it one of the most diagnostically challenging breast lesions. In contrast to most triple-negative breast cancers, FLMC follows a relatively indolent clinical course, though local recurrence is well documented, and because so few cases have been reported, no established treatment guidelines exist and the role of chemotherapy remains uncertain. We present the case of a 63-year-old woman recalled from routine screening digital breast tomosynthesis for an irregular, spiculated mass in the right breast, confirmed on biopsy to be FLMC, and treated with breast-conserving surgery and adjuvant radiation therapy without chemotherapy after two medical oncologists gave opposing recommendations regarding systemic treatment. She has remained without evidence of disease at two-year follow-up. This case adds to the limited literature on FLMC, supports surgery and radiation alone as a potentially effective treatment strategy in carefully selected patients, and highlights the importance of recognizing FLMC as a biologically distinct entity that should not be managed the same way as conventional triple-negative breast cancer, though longer-term follow-up is needed given the limited data on treatment outcomes for this rare tumor.

breast conservation↗
PubMed1979

Melanoma resembling spindle and epithelioid cell nevus.

Three cases of malignant melanoma resembling spindle and epithelioid cell nevus histologically are presented. Lesions having histologic features of spindle and epithelioid cell nevus (Spitz nevus or juvenile melanoma) at or after puberty should be regarded with caution, particularly when they are heavily melanized. Such lesions are in a histologic "gray zone" and may be malignant.

Adolescent↗
PubMed1977

Atypical fibroxanthoma of the skin: an ultrastructural study of two cases.

The ultrastructure of two atypical" fibroxanthomas of the skin was studied. The first lesion was a spindle cell tumor on light microscopy, which electron microscopy revealed was composed of undifferentiated mesenchymal cells. The second lesion had a highly pheomorphic appearance on both light and electron microscopy. It was formed by atypical histiocytic cells and abundant "typical" Langerhans cells. The second lesion was considered to be a proliferation either of atypical Langerhans cells or of histiocytes related to Langerhans cells. Thus, atypical fibroxanthoma of skin may not be a homogeneous entity but could be a group of mesenchymal proliferative lesion in a similar clinical setting.

Aged↗
PubMed1976

Hemangiopericytoma-like intranasal tumors. A clinicopathologic study of 23 cases.

The clinical, microscopic, and gross pathologic features of 23 cases of intranasal hemangiopericytoma-like tumors are reviewed and studied. When in the nasal cavity, these lesions often originated in a paranasal sinus and extended into the nasal cavity secondarily. They occurred most commonly in adults in the sixth and seventh decades of life; there was no significant sex predilection. Twenty-two of the 23 patients were Caucasian. These patients most commonly had symptoms of nasal obstruction and epistaxis. Clinically the lesions were generally thought to represent allergic polyps. Although appearing microscopically as non-differentiated spindle-cell neoplasms, these lesions showed little nuclear or cytoplasmic pleomorphism, minimal mitotic activity, and no necrosis or hemorrhage or other evidence of anaplasia found in malignant tumors. Follow-up data showed no evidence to suggest a malignant or biologically unpredictable lesion. Nineteen of 22 cases followed showed no recurrence regardless of the treatment; those that recurred did so locally. No metastasis or other form of aggressive behavior attributed to hemangiopericytomas in other anatomic locations was seen in this series. Another case, diagnosed as a malignant hemangiopericytoma of the nasal cavity, showed dissimilar and anaplastic histologic features. This case metastasized and is discussed, though not included in this study.

Adolescent↗
PubMed1976

Polypoid spindle-cell carcinoma (pleomorphic carcinoma). Report of a case occurring on tongue and review of the literature.

A case of spindle-cell carcinoma (pleomorphic carcinoma), a rare polypoid tumor of the tongue, is reported. The characteristic clinical, gross, and microscopic features of this peculiar lesion and its common sites of location are presented. Origin and pathogenesis of the sarcoma-like elements of the lesion are discussed and the literature on the subject is reviewed. Sites of apparent transition between the spindle cell elements of the tumor and the overlying epithelium were observed in light microscopic sections, and the electron micrographs of the spindle-cell portion of the lesion demonstrated the presence of tonofilaments and desmosomes in many tumor cells. These findings support the concept that this group of lesions are pleomorphic variants of squamous-cell carcinoma with predominantly spindle-cell pattern. Current evidence in the literature also suggests that metaplastic transformation of the tumor cells into mesenchymal elements may take place in some of these lesions, and that "metaplastic carcinoma" may probably be a more proper designation for them.

Aged↗
PubMed2025

The elevated expression of ORF75, a KSHV lytic gene, in Kaposi sarcoma lesions is driven by a GC-rich DNA cis element in its promoter region.

The spindle cells of Kaposi sarcoma (KS) lesions primarily express Kaposi sarcoma herpesvirus (KSHV) latent genes with minimal expression of lytic genes. However, recent transcriptome analyses of KS lesions have shown high expression of KSHV open reading frame (ORF) 75, which is considered a late lytic gene based on analyses in primary effusion lymphoma (PEL) lines. ORF75 encodes a pseudo-amidotransferase that is part of the viral tegument, acts as a suppressor of innate immunity, and is essential for viral lytic replication. We assessed a representative KS lesion by RNAscope and found that ORF75 RNA was expressed in the majority of latency-associated nuclear antigen (LANA)-expressing cells. Luciferase fusion reporter constructs of the ORF75 promoter were analyzed for factors potentially driving its expression in KS. The ORF75 promoter construct showed high basal transcriptional activity in vitro in endothelial cells, mediated by a proximal consensus specificity protein 1 (Sp1) (GGGGCGGGGC) element along with two distal CCAAT boxes. Sp proteins formed complexes with the proximal consensus Sp1 element to activate ORF75 promoter transcription. We also found evidence that a repressive factor or factors in B cells, but not endothelial or epithelial cells, interacted with more distal elements in the ORF75 promoter region to repress constitutive ORF75 expression in B cells. Alternate forms of Sp1 were found to accumulate during latency and showed substantial enrichment during viral lytic replication in PEL cells and infected endothelial cells, but their functional significance is unclear. We also found that ORF75 can in turn upregulate its own expression and that of other KSHV genes. Thus, while ORF75 acts primarily as a lytic gene in PEL cell lines, Sp proteins induce substantial constitutive ORF75 transcription in infected endothelial cells and this can account for its high expression in KS lesions.

Herpesvirus 8, Human↗
PubMed1979

Spindle cell lipoma of the orbit.

A 42-year-old woman had an orbital mass lesion removed surgically that proved histologically to be a spindle cell lipoma. To our knowledge, this is the first reported case of a spindle cell lipoma arising in the orbit. This specific type of lipoma occurs chiefly in male patients and is believed to affect the shoulder and posterior neck regions almost exclusively. Spindle cell lipomas, while having unusual features histologically, are benign and should not be mistaken for liposarcomas or other spindle cell soft-tissue tumors.

Adult↗
PubMed1979

Neurogenic sarcoma of the head and neck.

We discuss our experience in the diagnosis and management of seven cases of neurogenic sarcomas of the head and neck. These uncommon tumors, which affect all ages, arise most frequently from the brachial plexus, sympathetic chain, and the cranial nerves or their branches. An enlarging mass is the most common initially appearing symptom. Diagnosis may be difficult and rests heavily on gross evidence of a relationship between the tumor and its nerve of origin. Histologically, the lesions are composed of spindle cells with varying degrees of pleomorphism in a pattern that is frequently undistinguishable from fibrosarcomas. Ultrastructural studies may be helpful in establishing the neurogenic origin of these tumors. Neurogenic sarcoma of the head and neck has an extremely poor prognosis. Surgery remains the cornerstone of treatment, although radiotherapy is important for palliation.

Accessory Nerve↗
PubMed1977

Ischemic colitis and malignant atrophic papulosis.

A 37-year old male with a history of idiopathic nephrotic syndrome, hypertension, severe headaches and transient ischemic attacks developed ischemic colitis with stricture formation of the spelnic flexure. Eschemic changes were secondary to vascular lesions involving the middle colic artery and mulitple smaller arteries and arterioles. The vascular lesion is localized to the intimal layer with proliferation of spindle-shaped cells indentical to the gastrointestinal lesion of malignant atrophic papulosis (Degos' disease). The patient had no skin biopsy, or history of skin lesions. This case represents ischemic colitis in a patient with malignant atrophic papulosis with either absent or unrecognized skin lesions.

Adult↗
PubMed1976

Parosteal osteogenic sarcoma.

A clinicopathologic study of 79 patients with parosteal osteogenic sarcoma revealed that more females than males were affected and that most of the patients were in the second to the fourth decades of life. Sixty-eight percent of the patients had involvement of the posterior aspect of the lower femoral shaft. The roentgenogram characteristically showed a large, dense lobulated mass attached by a broad base to the underlying bone but with no involvement of bone itself. Microscopically, the tumor presented as well-formed bands of osteoid within a hypocellular spindle cell stroma. Seven of the lesions had foci of high-grade osteogenic sarcoma within an otherwise typical parosteal osteogenic sarcoma. Six of the 79 lesions showed involvement of the medullary cavity at surgery. Thirty-one patients had excision as their initial treatment; four of the 31 required nv further therapy. Ten patients had resection initially; three of the ten had recurrence. Only four of 27 patients who underwent amputation initially developed pulmonary metastasis. The presence of histologically "active" tumor and medullary involvement seemed to affect the prognosis adversely. Our data indicate that complete radical removal of the tumor is the treatment of choice, with resection when feasible and amputation when necessary.

Amputation, Surgical↗
PubMed2026

Spitz tumours: Current insights and challenges in diagnosis and management.

Spitz tumours are a distinct subtype of melanocytic lesions composed of epithelioid and/or spindled cells. They comprise Spitz naevi (SN), atypical Spitz tumours/Spitz melanocytoma (AST), and Spitz melanoma (SM). According to the 5th WHO Classification, SM is defined by the co-occurrence of spitzoid morphology and a Spitz-defining genomic alteration, typically a kinase fusion or HRAS mutation, making it a molecularly distinct entity. Molecularly confirmed SM is exceedingly rare, usually occurs in younger adults, and appears to demonstrate a more favorable clinical course than spitzoid melanoma driven by BRAF or NRAS mutations, although robust comparative data remain limited. Dermoscopy may raise clinical suspicion based on characteristic patterns and remains an important first-line diagnostic tool; however, it cannot reliably distinguish AST from SM. In addition, Spitz tumours may exhibit overlapping histopathological features, further complicating their differentiation. In particular, the distinction between ASTs and SMs is often challenging. Immunohistochemical and molecular analyses, particularly next-generation sequencing (NGS), play a crucial role in resolving diagnostically challenging Spitz tumours and in differentiating true Spitz tumours from their morphological mimics. Furthermore, NGS contributes to improved risk stratification and has revealed high-risk genomic alterations associated with progression. Increased application of molecular techniques is expected to refine prognostic assessment and support individualized management strategies.

Journal Article↗
PubMedDate not supplied

Fibrous xanthoma of the frontal sinus.

Frontal sinus mucocele was our preoperative diagnosis in a patient who had frontal swelling and downward displacement of the eye. Supporting this were typical roentgenogram changes, a long history of asthma, pansinusitis, and previous multiple-polypectomy surgery. Frontal sinus exploration revealed a locally eroding lesion. It was composed of spindled cells and lipid-laden histiocytes with a pattern of fibrous xanthoma and was treated conservatively. It should not be confused with true malignancies such as fibrous histiocytoma having a similar histopathologic appearance and requiring more aggressive surgical treatment.

Fibroma↗
PubMed1976

Ultrastructure of spindle cell squamous carcinoma.

A 63-year-old white male presented with a nine-month history of a nontender ulcerated lesion on his ear. Light microscopy demonstrated a moderately well circumscribed lesion in the dermis which abutted upon epidermis. There was no evidence of continuity between the tumor and overlying epithelium. The tumor was very cellular with an admixture of cells - spindle, polyhedral and bizarre giant cells. Mitotoc figures were abundant and frequently abnormal. We interpreted this lesion to have the clinical and pathologic features of an atypical fibroxanthoma (AFX). Ultrastructure, however, showed abundant tonofilaments and desmosomes indicative of an epithelial origin and therefore most consistent with a spindle cell squamous carcinoma (SCSC). It is urged that, when possible, electron microscopy be performed on problematic cases diagnosed either as an AFX or spindle cell squamous carcinoma since it is the most valid basis on which a correct diagnosis can be made.

Carcinoma, Squamous Cell↗
PubMed1979

[Differential diagnosis of a metastasizing adamantinoma of the tibia and fibula (author's transl)].

A case of an adamantinoma of the tibia and fibula is described in a 10-year-old girl who died after 17 years with pulmonary metastasis. For a long time, an exact diagnosis couldn't be made. Histologically the lesion was called chronic osteomyelitis (Brodie's abscess), synovial sarcoma and spindle cell sarcoma. The revision of the histological sections revealed a fibrous dysplasia-like pattern in the first lesion of the tibia with tiny foci of an epitheloid-like pattern. This had could be the key to the right diagnosis, because sometimes adamantinoma of long bones is associated with a fibrous dysplasia-like pattern. The reason for misinterpretation of the histological features is seen in the typical variable histological pattern of this tumor (basaloids, squamoid, spindled and tubular pattern). In this connection is refered to the necessity to correlate the histological feature with the X-ray in making diagnosis in bone pathology.

Adult↗
PubMed1976

Endobronchial plasma cell granuloma (xanthomatous pseudotumor); a light and electron microscopic study.

Plasma cell granuloma (xanthomatous pseudotumor) is polymorphic at light as well as electron microscopic examination. At light microscopy the endobronchial variant of this entity was rich in plasma cells and interwoven, whorl-like, or concentrically arranged spindle cells. Foamy histiocytes and macrophages usually abundant in the intrapulmonary variant were rare. At electron microscopy particles 20 to 50 nm. in size were found at the bronchial mucosal surface but not elsewhere in the lesion. Plasma cells near the bronchial surface contained cytoplasmic fibrils, mitochondria with concentric cristae, and inclusions that bore a close resemblance to adjacent extracellular crystallized hemoglobin. Those in the center of the lesion ordinary round inclusions and none of the other changes. Spindle cells in the interlaced areas were mostly fibroblasts or myofibroblasts, whereas those whorled around capillaries resembled pericytes with basement membranes and nuxes-like intercellular junctions. Year rings like multilayered basal laminae were frequently present between the pericyte-like cells and the endothelial cells of the capillaries. The ultrastructure of plasma cell granuloma, like the histologic and clinical aspects, differs from that of sclerosing hemangioma, pseudolymphoma, and malignant plasma cell tumor affecting the lung.

Adult↗