scholarly journals Primary Leiomyosarcoma of the Penis: A Review and Update of the Literature

2019 ◽  
pp. 43-51
Author(s):  
Anthony Kodzo-Grey Venyo

Less than 50 cases of Primary Leiomyosarcomas of the Penis (PLOP) have been reported despite this PLOPs are the second most common sarcomas of the penis. The usual site for the development of PLOP is the shaft or base of the penis but the malignancy can affect any site of the penis. PLOPs are likely to be superficially located above the tunica albuginea in comparison with deep seated PLOPs. Superficial PLOPs may be asymptomatic or they may present as lumps, ulcers or nodules on the penis. Deep PLOPs may manifest with dysuria and difficulty with micturition when they compress the urethra. Diagnosis of PLOP is based upon the pathology examination features of specimens of the penile lesion that show interlacing fascicles of spindled-cells that have abundant eosinophilic cytoplasm, focal juxta-nuclear vacuoles and blunt ended nuclei and moderate to severe nuclear atypia, rarely atypical multi-nucleated giant cells and focal necrosis tend to be seen. There may be evidence of many mitoses. Immunohistochemistry studies of PLOP tend to show tumor cells that exhibit positive staining for desmin, muscle specific-actin, and alpha smooth muscle actin. Most often superficial PLOPs tend to be treated by wide complete excision of the tumor with tumor free and clear surgical resection margins. Deep seated PLOPs have also been treated by complete excision in various forms depending upon the site and size of the tumor and some of these have included Wide excision, partial penectomy and total penectomy alone and sometimes with adjuvant chemotherapy/radiotherapy. Some cases of deep PLOPs tend to be associated with the development of recurrences and metastases as well as death of the patient and hence deep PLOPs are aggressive tumors. Some people are of the opinion that chemotherapy and radiotherapy are not effective for the adjuvant treatment of PLOP. For this reason it would appear that there is need for the development of new chemotherapy medicaments that would effectively destroy PLOP tumor cells. There is need to undertake a global multi-center treatment trial of immunotherapy, chemotherapy and radiotherapy in order to streamline the treatment of PLOPs especially deep PLOPs.

2016 ◽  
Vol 144 (9-10) ◽  
pp. 535-540
Author(s):  
Aguida Henriques ◽  
Manuela Vidal ◽  
Clarissa Gurgel ◽  
Sanyra Rocha ◽  
Braulio Carneiro ◽  
...  

Introduction. Epithelioid hemangioma is an uncommon benign vasoproliferative neoplasm that usually manifests as multiple red nodules in middle-aged adults. Case Outline. A 52-year-old male patient presented with a one-year history of a nodular lesion in the left buccal mucosa measuring 3 cm. The clinical hypothesis was lipoma. An excisional biopsy revealed a circumscribed lesion composed of lobules of vessels with perceptible or poor lumina, associated with a prominent inflammatory infiltrate consisting of eosinophils, histiocytes and chronic inflammatory cells. The endothelial cells composing the lesion had an epithelioid morphology and contained abundant eosinophilic cytoplasm. Immunohistochemistry for CD34, factor VIII, collagen IV, alpha-smooth muscle actin, and mast cells, as well as histochemical staining with Weigert?s orcein were performed. Conclusion. Vascular proliferations of soft tissues are a diverse and morphologically complex group of lesions that are difficult to diagnose. This report presents a case of oral epithelioid hemangioma, highlighting relevant morphological and immunohistochemical features that could help distinguish this condition from other neoplasms.


F1000Research ◽  
2016 ◽  
Vol 5 ◽  
pp. 2905 ◽  
Author(s):  
Sunil Munakomi ◽  
Pramod Chaudhary

Herein we report a rare case of a giant myopericytoma presenting in a 16-year-old girl as a slowly progressive swelling involving the scalp in the occipital region. It was managed by complete excision. Histological examination of the lesion revealed  spindle-shaped cells forming characteristic rosettes around the blood vessels, and positive staining with smooth muscle actin.


2018 ◽  
Vol 12 ◽  
pp. 117955491881353
Author(s):  
Yanling Zhang ◽  
Heng Tang ◽  
Huaiyuan Hu ◽  
Xiang Yong

Primary leiomyomas of the thyroid are very rare. We here report a case of a 53-year-old woman with a painless mass at the right thyroid, revealed by physical examination. The patient underwent a lobectomy. Frozen sections showed a spindle cell tumor of the thyroid gland. The nuclei of some of the tumor cells were obviously enlarged and deeply stained. Pseudocapsule invasion was observed in small foci. Samples showed neither mitosis nor necrosis and the nature of the tumor was difficult to determine. Paraffin sections showed a well-circumscribed nodular composed of intersecting fascicles of spindled to slightly epithelioid cells with eosinophilic cytoplasm and blunt-ended, cigar-shaped nuclei. We observed no significant nuclear atypia, mitotsis, or necrosis. Immunohistochemical staining showed the tumor cells to be positive for α-smooth muscle actin and h-caldesmon but negative for TG, TTF1, PAX8, S-100, CT, CK, and CD34. The ki-67 index was very low (<1%). Primary thyroid leiomyoma is rare and difficult to diagnose using frozen sections. Diagnosis requires immunohistochemical staining. Leiomyoma may be mistaken for other thyroid tumors also characterized by spindle cells.


1998 ◽  
Vol 112 (4) ◽  
pp. 373-376 ◽  
Author(s):  
Luis V. Victoria ◽  
Henry T. Hoffman ◽  
Robert A. Robinson

AbstractGranular cell tumour (GCT) is a benign tumour with abundant eosinophilic cytoplasm filled with granules of varying sizes. These granules are the defining characteristic of the GCT and are believed to represent lysosomes in varying stages of fragmentation. The commonly used term granular cell myoblastoma, found in the older literature, is a misnomer because the tumour is clearly not of muscle origin. Among the major theories of origin, some support the tumour's derivation from neuronal tissue, histiocytes, fibroblast or Schwann cells. In the larynx, pseudoepitheliomatous hyperplasia may predispose to confuse the GCT with squamous cell carcinoma.The most common region of GCT is in the head and neck, accounting for approximately 30 to 50 per cent of all lesions. The larynx is relatively an uncommon location for these tumours, accounting for approximately three to 10 per cent of the reported cases.Affected patients typically present with persistent hoarseness, stridor, haemoptysis, dysphagia, and otalgia, but the tumour may be asymptomatic and be discovered only incidentally during a routine examination.Complete excision with an attempt to maintain normal stuctures generally results in cure.We present the case of a patient with typical features of a GCT of the larynx. The gross appearance, histopathology and brief discussion of the current literature are also presented.


2002 ◽  
Vol 39 (2) ◽  
pp. 281-285 ◽  
Author(s):  
M. Y. Gulbahar ◽  
I. Alkan ◽  
L. Aslan ◽  
I. Golen

A 4-year-old native-breed cow had a mass with wide areas of ulceration and hemorrhage at the base of the tail at the same level as the vulva. The tumor was 19 X 13 X 11 cm, appeared red-brown, and was firm to hard, with gritty areas apparent on cut surface. Histologically, the tumor mass was composed of multilayered epithelial cells forming glandular structures with occasional apical blebs and rare solidly packed cells in nests. The stroma included fibrous connective tissue, scattered or periglandular sheets of spindle-shaped cells resembling myoepithelium, several cartilaginous formations, and numerous irregular islands of mineralized osteoid, well-formed bone trabeculae lined by osteoblasts, and many osteoclast-like multinucleated giant cells among or near the neoplastic epithelium. Immunohistochemically, the neoplastic epithelium was positive for pan-cytokeratin (AE1/AE2) and cytokeratin 19 but was negative for cytokeratin 18. Spindle-shaped cells were stained with alpha smooth muscle actin (αSMA) and to a lesser extent vimentin antibodies. The cells of osteogenic lineage and spindle cells closely associated with the osteoid showed strong immunostaining for vimentin but not for αSMA. Immunostaining for neuron-specific enolase and S100 protein was not observed in any component of the tumor mass. These findings suggested that the origin of bone formation was undifferentiated mesenchymal cells with osteogenic potential.


2021 ◽  
Vol 156 (Supplement_1) ◽  
pp. S81-S81
Author(s):  
E Shkolnik ◽  
D Cai

Abstract Introduction/Objective Epithelioid sarcoma (ES) is a rare, malignant mesenchymal neoplasm that has a known tendency for local recurrence, regional lymph node involvement, and distant metastases. Two histologic variants have been recognized: classic ES also known as the distal type, and proximal-type ES (PES). The classic ES is common in young adults. It occurs more frequently in the distal upper extremities followed by the distal lower limbs and has a male prevalence of 2:1. Conversely, PES commonly involves deep tissues in the pelvic region, including the genital area. It tends to occur in older patients and follows a more aggressive clinical course. In the female genital tract, PES occurs most frequently in the vulva. The incidence of primary sarcoma of the vulva accounts for 1.5-5% of all malignant tumors, making PES a very rare incidence. Methods/Case Report Here we report a 60-year-old female diagnosed with vulvar epithelioid sarcoma treated with a right radical vulvectomy and bilateral inguinal lymph node dissection in 2008. In 2017, further surgery and adjuvant radiation were given for local recurrence. In 2020, the patient developed left hip pain and was found to have an expansile lytic lesion in the left proximal femur. Extensive resection was performed. Grossly the vulvar lesion was nodular with diffuse hemorrhage, degeneration, and necrosis. Microscopically, the tumor cells had large vesicular nuclei, prominent nucleoli, and abundant eosinophilic cytoplasm. Histologically, the morphology of the tumor cells are similar for the primary vulvar specimen and the bone metastases. Immunohistochemically, the tumor cells are positive for vimentin, GATA, FLI-1, SMA, SMHC, partially positive for CAM5.2, AE1/AE3, CD31, and CD163. Immunohistochemistry was negative for CDX2, CD56, S-100, TTF-1, CK5/6, CK20, P40, mammoglobin, MOC31, ER, CK7, CK903, HMB45, PAX8. A Ki-67 proliferative index was around 30-40%. NGS molecular testing detected a SMARCB1 mutation with loss of exons 1-3 and exons 7-9 supporting the diagnosis of epithelioid sarcoma. Results (if a Case Study enter NA) NA Conclusion In summary, we report a case of PES of the vulva in a 60 year old female. Grossly, the lesion was nodular with histology showing large vesicular nuclei, prominent nucleoli, and abundant eosinophilic cytoplasm. It showed loss of INI1/SMARCB1 nuclear expression. The patient is receiving further adjuvant treatment and shows no new metastases.


Author(s):  
Kusum Yadav ◽  
Jitendra Singh Nigam ◽  
Anshul Singh ◽  
Vatsala Misra

Phyllodes tumour is a rare tumour of the breast constituting less than 1% of all breast tumours. Malignant Phyllodes Tumour (MPT) accounts for only 10-30% of all phyllodes tumours. Heterologous sarcomatous differentiation in a MPT is an infrequent phenomenon, with the cases reported showing differentiation mostly towards liposarcoma, fibrosarcoma, angiosarcoma, osteosarcoma, or chondrosarcoma. MPTs with rhabdomyosarcomatous differentiation are scarcely seen with only three confirmed cases documented till date to the best of the knowledge after a thorough search of literature. Here, authors present a case of 45-year-old female who presented with a well-defined rapidly growing lump in the right breast for last one year. A core needle biopsy performed showed a sarcomatous picture on histology. Complete excision was subsequently done. On microscopy, most of the areas showed fibrosarcomatous changes with frequent mitoses. Some of the foci showed large pleomorphic cells in diffuse sheets that were polygonal with densely abundant eosinophilic cytoplasm and vesicular nucleus with prominent nucleoli (rhabdomyoblasts). Myogenin was diffusely positive on Immunohistochemistry (IHC). A diagnosis of MPT with rhabdomyosarcomatous differentiation was made. This case is reported here for its unusual presentation and to make pathologists aware of this rare heterologous differentiation of MPT.


Author(s):  
Xinglong Geng ◽  
Hongze Chen ◽  
Liang Zhao ◽  
Jisheng Hu ◽  
Wenbo Yang ◽  
...  

Pancreatic ductal adenocarcinoma (PDAC) is a highly lethal disease that typically features a dramatic desmoplastic reaction, especially fibroblasts. The roles of cancer-associated fibroblasts (CAFs) in PDAC have received more attention in recent years. As increasing evidence suggests the heterogeneity of CAFs in PDAC, different CAF subtypes have been shown to support tumor growth, while others suppress cancer proliferation. Myofibrotic CAFs (myCAFs) show alpha-smooth muscle actin (α-SMA)high interleukin-6 (IL-6)low myofibroblastic features, are activated by direct contact with tumor cells, and are located in the periglandular region. Inflammatory CAFs (iCAFs) show α-SMAlow IL-6high inflammatory features, are activated by paracrine factors secreted from tumor cells, and are located away from cancer cells. Antigen-presenting CAFs (apCAFs) show major histocompatibility complex II (MHC II) family genes that are highly expressed. CAFs have also been gradually explored as diagnostic and prognostic markers in pancreatic cancer. Targeted therapy of CAFs in PDAC has gradually attracted attention. With the deepening of related studies, some meaningful positive and negative results have surfaced, and CAFs may be the key to unlocking the door to pancreatic cancer treatment. Our review summarizes recent advances in the heterogeneity, function, and markers of CAFs in pancreatic cancer, as well as research and treatment targeting CAFs in pancreatic cancer.


2018 ◽  
Vol 8 (2) ◽  
pp. 7
Author(s):  
Konstantinos Kosmas ◽  
Anna Tsonou ◽  
Georgia Mitropoulou ◽  
Anastasia Mandilara ◽  
Olga Papadopoulou ◽  
...  

Malignant melanoma (MM) is a malignant melanocytic neoplasm that occurs mainly in the skin but it can also involve any tissue. It has the capacity to metastasize widely and quickly to various sites without any intermediate stops, sometimes many years after treatment of the primary tumor. It is almost impossible to predict which organ system will be invaded by melanoma from a given primary site. We report the cytomorphologic and immunocytochemical findings of a male patient with isolated pleural metastasis of MM without pulmonary parenchymal metastatic involvement after 10 years of progression-free survival. Pleural fluid cytology revealed epithelioid cells of variable sizes and configuration isolated or in clusters with abnormal hyperchromatic nuclei, irregularly-shaped nucleoli, abundant eosinophilic cytoplasm, multinucleated giant cells, intranuclear cytoplamic inclusions as well as many cytoplasmic melanin pigmented tumor cells. Immunocytochemical markers for melanoma HMB-45 and S-100 were positive. Metastasis of MM to pleural fluid is rare and diagnosing the disease by cytology is challenging and requires medical expertise as well as knowledge of clinical context and immunocytochemical staining evaluation.   


2020 ◽  
Vol 28 (6) ◽  
pp. 691-695 ◽  
Author(s):  
Irene Y. Chen ◽  
Bushra G. Fazili ◽  
Xiaoyan Liao

Glomus tumor is a rare mesenchymal neoplasm originating from the modified smooth muscle cells of the glomus body. Primary colonic glomus tumor is extremely rare with only 5 cases published in the English literature. In this article, we report the sixth case of primary colonic glomus tumor in a 50-year-old female with no significant past medical history who presented with routine screening colonoscopy. The entire colon was endoscopically unremarkable except an incidental 6-mm sessile polyp located in the descending colon. Biopsy showed a densely cellular neoplasm composed of small, bland, slightly spindled to predominantly epithelioid cells with clear to eosinophilic cytoplasm arranged in nests and sheets. The tumor cells were interspersed with slit-like thin-walled vessels and scattered short nerve bundles. Immunohistochemically, the tumor cells were positive for smooth muscle actin, h-caldesmon, and CD34 (focal), but completely negative for HMB45, S100, EMA, desmin, DOG-1, and CD117. The histologic features and immunohistochemical profile supported a diagnosis of primary colonic glomus tumor. The patient was asymptomatic and disease free after the procedure.


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