scholarly journals Case Report: Myelodysplastic syndrome- associated myeloid sarcoma: an unusual clinical presentation of a rare disease

F1000Research ◽  
2016 ◽  
Vol 5 ◽  
pp. 128 ◽  
Author(s):  
Emoke Horvath ◽  
Smaranda Demian ◽  
Elod Nagy

Myeloid sarcoma results from the extramedullary homing and proliferation of immature myeloid precursors. We present the timeline, events and diagnostic pitfalls related to a 66 year-old male patient’s case, admitted to the Hematology Clinic for pancytopenia, fever, weight loss and fatigue. The severe cytopenia and the few blasts observed in his blood smear indicated a bone marrow biopsy. The bone marrow showed hypercellularity and multilineage dysplasia with the presence of 15% myeloblasts. After the biopsy, he promptly developed paraplegia and nuclear magnetic resonance revealed an epidural tumour which was then resected.In the epidural tumour mass blast-like, round cells were observed with a complex immunophenotype, characterized by myeloperoxidase, CD117, CD15, CD99, leucocyte common antigen positivity and a high Ki-67 proliferation index. Considering the main differential diagnostic issues, the final diagnosis was stated as myelodysplastic syndrome-associated myeloid sarcoma. The prognosis was unfavourable, the bone marrow was quickly invaded by proliferating blast cells, and despite chemotherapy attempts, the patient died.

2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S97-S97
Author(s):  
A Herrmann ◽  
B Mai ◽  
S Elzamly ◽  
A Wahed ◽  
A Nguyen ◽  
...  

Abstract Introduction/Objective A 46-year-old female presented with severe back pain associated with progressive bilateral lower extremity weakness and paresthesia, urinary retention, and constipation. Computed tomography revealed a retroperitoneal mass encasing the right psoas muscle, obstructing the right kidney, and extending to the thoracolumbar region resulting in severe spinal compression. An epidural tumor resection was subsequently performed at an outside hospital. Methods Histological sections showed sheets of blastoid neoplastic cells with intermediate to large nuclei, irregular membranes, fine chromatin, and prominent nucleoli. Immunohistochemical stains showed that these cells were positive for CD43, CD79a (weak, focal), BCL2, C-MYC, and PAX5 (weak, focal) and negative for CD10, CD20, CD30, ALK1, BCL6, MUM1, and Tdt. The Ki-67 proliferation index was 75-80%. With this immunophenotype, this patient was diagnosed with a high grade B-cell lymphoma and transferred to our institution for further work-up. On review of the slides, further immunohistochemical testing was requested which revealed positivity for CD117 and myeloperoxidase (MPO). Results The overall morphological and immunophenotypical features are most compatible with myeloid sarcoma (MS) with aberrant expression of B-cell markers and this patient’s diagnosis was amended. Interestingly, the patient’s bone marrow examination only showed 2% myeloblasts with left shifted granulocytosis and concurrent fluorescence in situ hybridization (FISH) studies were negative. Conclusion A literature review showed that 40-50% of MS are misdiagnosed as lymphoma. MS can frequently stain with B-cell or T-cell markers, as seen in this case, which makes it challenging for an accurate diagnosis and sub- classification. In addition, our case is interesting in that there was only extramedullary presentation without bone marrow involvement. Typically, MS develops after the diagnosis of acute myeloid leukemia (AML) with an incidence of 3–5% after AML. It can also manifest de novo in healthy patients, who then go on to develop AML months to years later. Therefore, this patient will require close follow-up.


2017 ◽  
Vol 65 (1) ◽  
pp. 89-95
Author(s):  
Gustavo A. Ramírez ◽  
Lorenzo Ressel ◽  
Jaume Altimira ◽  
Miquel Vilafranca

A 13-year-old male cat presented with an ill-defined mass in the rostral mandible causing destruction and loss of alveolar bone. Microscopically, the mass consisted of cords or islands of benign odontogenic epithelium and a malignant, pleomorphic spindle-shaped cell component with dysplastic dentine formation. Immunohistochemically, neoplastic mesenchymal cells proved to be strongly positive for vimentin and negative for cytokeratins, desmin, actin and S100 protein; the Ki67 proliferation index was high. Morphological and immunohistochemical features largely overlap those reported for ameloblastic fibrodentinosarcoma, an uncommon histologic subtype of odontogenic sarcoma recognised in humans but no reported previously in animals. Ki-67 expression assessment may help to discriminate between malignant and benign forms of odontogenic tumours but the final diagnosis is mainly morphological.


2021 ◽  
Author(s):  
Yan Tan ◽  
Xinyi Wang ◽  
Guifang Yang ◽  
Lan Liu ◽  
Jun Fang ◽  
...  

Abstract BackgroundGlomus tumors are exceedingly rare tumors arising from the normal glomus body. Only a very few cases of glomus tumors located in the small intestine were reported to date. Here, we present a case of glomus tumor of the jejunum in a 30-year-old female patient, and discuss its clinical, imaging, and pathologic features.Case presentationA 30-year-old female referred to our hospital with the chief complain of melena and fatigue for 3 weeks. Oral single-balloon enteroscopy discovered a 2.0*2.0cm tumor without epithelial lining in the jejunum. The patient then underwent partial enterectomy. HE stain illustrated that the tumor was interspersed with congestive capillaries of various size, and admixed with smooth muscle bundles. Immunohistochemical study showed that the tumor cells were strongly positive for SMA and collagen type Ⅳ. The Ki-67 proliferation index was less than 1% and mitotic activity is very low (about 1/50 HPF). The case was finally diagnosed as benign glomus tumor.ConclusionsGlomus tumors arising from the small intestine are extremely rare. The final diagnosis is made by histological and immunohistochemical examination. Although there is no standardized management pathway for these patients, early diagnosis and treatment are important for a good prognosis.


2020 ◽  
Vol 1 (4) ◽  
Author(s):  
Nohelia Rojas Ferrer ◽  
María D Berenguer Romero ◽  
M. Cano Medina ◽  
Amparo de Fez Satores ◽  
Jorge Escandón Álvarez

Myeloid sarcoma, granulocytic sarcoma, chloroma or extramedullary myeloid leukemia, is an unusual tumor, with a dire prognosis. Up today, the clinical diagnosis continues to be extremely difficult due to the nonspecific symptoms. Histology, immunohistochemistry, and flow cytometry are valuable in the diagnose of the tumor, as well as in the differential diagnosis from multiple entities with different prognoses and treatments. We present 61-year-old men who presented with three days evolution abdominal pain, vomiting and abdominal distension. A diagnosis of adenocarcinoma was presumed. A final diagnosis of intestinal myeloid sarcoma without evidence of bone marrow infiltration was established. A review of the most relevant aspects of this disease is presented.


2019 ◽  
Vol 9 (2) ◽  
pp. 1599-1603
Author(s):  
Pallavi Srivastava ◽  
Saumya Shukla ◽  
Nuzhat Husain ◽  
Priyanka Sameer

The neuroendocrine tumor of extrahepatic biliary tract is a rare neoplasm of the gastrointestinal tract. We present a case of 16 year old male presenting with epigastric pain and jaundice with a well-defined lesion in common hepatic duct on imaging. The patient underwent tumor resection. Histopathology examination revealed thickened common hepatic duct infiltrated by tumor cells with expression of Pan CK, Synaptophysin & Chromogranin with Ki- 67 proliferation index of 5%. The final diagnosis of Neuroendocrine tumor of Common Hepatic Duct, grade 2 was rendered. The patient showed no recurrence to date without intravenous chemotherapy.


Author(s):  
N. N. Tupitsyn ◽  
V. A. Mkrtchyan ◽  
A. D. Palladina ◽  
I. K. Vorotnikov

The innate immunity system plays an important role in antitumor protection, and more and more attention has been paid to its study recent years. However, the interrelation of the effect or subpopulations of the cells of the innate immunity in bone marrow with clinical parameters is poorly studied. The paper presents data on the composition of innate immune cells in the bone marrow of 64 patients with operable breast cancer, as well as 10 women with benign processes in the mammary gland. As result a significant correlation between the molecular subtype of cancer and the level of B1- lymphocytes was identified. Breast cancer patients levels of NK cells (CD56 + CD3- and CD16 + CD3-) in the bone marrow were significantly higher in patients with low proliferative activity (Ki-67 less than 20%), compared to patients having a high tumor proliferation index. Populations of NK cells were interrelated with erythropoiesis in patients with breast cancer and were significantly higher in cases of reduced basophilic and polychromatophilicnormoblasts.


Author(s):  
G.S. Starodub ◽  
N.V. Goryainova ◽  
T.P. Perekhrestenko ◽  
O.V. Basova ◽  
N.M. Tretiak ◽  
...  

Patients with MDS RAEB II were examined. The decrease in dynamics of intracellular K³-67 protein expression was determined in patients with MDS RAEB II with positive response to chemotherapy, and the increase in proliferative activity of haematopoietic cells of peripheral blood (PB) and bone marrow (BM) was determined in patients with MDS RAEB II in transformation and acute myeloid leukaemia (AML) after MDS.


2021 ◽  
Author(s):  
Yan Tan ◽  
Xinyi Wang ◽  
Guifang Yang ◽  
Lan Liu ◽  
Jun Fang ◽  
...  

Abstract Background: Glomus tumors are exceedingly rare tumors arising from the normal glomus body. Only a very few cases of glomus tumors located in the small intestine were reported to date. Here, we present a case of glomus tumor of the jejunum in a 30-year-old female patient, and discuss its clinical, imaging, and pathologic features.Case presentation: A 30-year-old female referred to our hospital with the chief complain of melena and fatigue for 3 weeks. Oral single-balloon enteroscopy discovered a 2.0*2.0cm tumor without epithelial lining in the jejunum. The patient then underwent partial enterectomy. HE stain illustrated that the tumor was interspersed with congestive capillaries of various size, and admixed with smooth muscle bundles. Immunohistochemical study showed that the tumor cells were strongly positive for SMA and collagen type Ⅳ. The Ki-67 proliferation index was less than 1% and mitotic activity is very low (about 1/50 HPF). The case was finally diagnosed as benign glomus tumor. Conclusion: Glomus tumors arising from the small intestine are extremely rare. The final diagnosis is made by histological and immunohistochemical examination. Although there is no standardized management pathway for these patients, early diagnosis and treatment are important for a good prognosis.


2017 ◽  
Vol 11 (1-2) ◽  
pp. 66 ◽  
Author(s):  
Mary K. Sweeney ◽  
Soroush Rais-Bahrami ◽  
Jennifer Gordetsky

Inverted urothelial papilloma (IUP) is a rare, non-invasive endophytic lesion that accounts for 1‒2% of urothelial tumours. On cystoscopy, IUP appears as a pedunculated/papillary mass with a smooth surface. On microscopy, IUP has an endophytic growth pattern with the bulk of the tumour covered by a superficial layer of urothelium, which can be hyperplastic or attenuated. The cytology should be bland, with uniform, spindled cells arranged in anastomosing trabeculae and cords with peripheral palisading of basaloid cells. Exophytic papillae and mitotic activity should be absent or focal. Pseudoglandular spaces and squamous metaplasia may also be present. There are distinct molecular differences between IUP and urothelial carcinoma (UC). IUP rarely has mutations of FGFR3, homozygous loss of 9p21, or gain of chromosomes 3, 7, and 17, whereas these mutations are frequently seen in UC. In addition, IUP is much less likely to have TERT mutations compared to UC. Immunohistochemistry can also be helpful in distinguishing the two entities as IUP is typically negative for CK20 and has a low Ki-67 proliferation index. Positivity for p53 may be seen in a minority of IUP. IUP can recur and be seen in association with UC. Distinguishing IUP from UC can be difficult due to the similarity between the two entities both on cystoscopy and histology, as up to 25% of UCs will also have inverted growth. Given the morphologic variants of IUP and UC, it is possible for a diagnostic error to occur, which can significantly impact patient management.


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