MyMedR

Displaying publications 1 - 20 of 41 in total

Abstract:

Sort:

Malignant peripheral nerve sheath tumour with perineurial differentiation and hyaline eosinophilic globules (thanatosomes): A rare tumour

Abdelzaher E, Elwany A, Amr SA

Malays J Pathol, 2018 Dec;40(3):355-358.
PMID: 30580369

Malignant peripheral nerve sheath tumour (MPNST) with perineurial differentiation is a rare variant of MPNST. The pathological features and clinical significance of this variant remain to be characterised. We reported the clinicoradiological and pathological features of a case of recurrent right arm mass related to the ulnar nerve in a 42-year-old female patient. On pathological examination, the tumour showed dual features of conventional and perineurial MPNST which was proven by positive immunostaining for S-100 and EMA. The pathological diagnosis was MPNST with perineurial differentiation. In addition, a peculiar and rare finding of intracytoplasmic eosinophilic hyaline globules (thanatosomes) within tumour cells is reported. We document a rare tumour with hybrid features between conventional and perineurial MPNSTs. Further studies are needed to establish its biological behaviour.

Matched MeSH terms: Soft Tissue Neoplasms/metabolism; Soft Tissue Neoplasms/pathology*; Soft Tissue Neoplasms/surgery
Giant cell tumor of the tendon sheath in the knee of an 11-year-old girl

Abdullah A, Abdullah S, Haflah NH, Ibrahim S

J Chin Med Assoc, 2010 Jan;73(1):47-51.
PMID: 20103492

Giant cell tumors are commonly found over the flexor tendon sheath of the hand and wrist. However, giant cell tumors in the knee joint are rare, especially in children. We report an interesting case of an 11-year-old girl who presented with a painful lump on her right knee that enlarged over time. Clinically, she had fullness over the anterolateral part of her knee. Magnetic resonance imaging revealed an encapsulated mass inferior to the patella. The tumor measured 3 x 3.5 x 1.5 cm. Histopathological findings confirmed that it was a tenosynovial giant cell tumor. Because of initial mild symptoms, there was a delay of 2 years from the initial symptoms until tumor excision. Her follow-up period was 35 months, and her health to date is excellent with no recurrence. We believe that reporting this rare case will help clinicians update their knowledge on possible causes of lumps in the knee, and avoid diagnostic delay. It could also prove to be beneficial in arriving at a diagnosis in future cases.

Matched MeSH terms: Soft Tissue Neoplasms/diagnosis*; Soft Tissue Neoplasms/pathology; Soft Tissue Neoplasms/surgery
Fulltext Rapidly Growing Huge Lower Back Malignant Peripheral Nerve Sheath Tumour

Abuzarifa N, Al-Chalabi MMM, Wan Sulaiman WA

Cureus, 2021 Mar 05;13(3):e13712.
PMID: 33842103 DOI: 10.7759/cureus.13712

Malignant peripheral nerve sheath tumours (also called neurofibrosarcomas) are a rare, highly aggressive soft tissue sarcomas that arise from the peripheral nerves or cells associated with the nerve sheath, such as Schwann cells, peri-neural cells and fibroblasts. It is representing 10% of all soft tissue sarcomas in which it is considered as an extremely rare malignancy, especially in patients with neurofibromatosis type I. In the general population, it affects approximately 1 in 100,000 people. This article is reporting a 56-year-old Malay female patient who is a known case of neurofibromatosis type I for 20 years, presented with a lower back, pruritic, gradually increasing swelling during the last five months. Last month before the presentation, the lesion rapidly grows, reaching a size of (15×15 cm), accompanied by foul-smelling discharge and pain exacerbated with movement. Although no history of preceding trauma or accident, the mass bleeds within contact. In conclusion, only a few cases of giant malignant peripheral nerve sheath tumours reported in the literature describing its location and growth progression. We present a massive, extremely rapid growth of cutaneous exophytic malignant peripheral nerve sheath tumours over the lower back.

Matched MeSH terms: Soft Tissue Neoplasms
Li-Fraumeni syndrome in a Malaysian kindred

Ariffin H, Martel-Planche G, Daud SS, Ibrahim K, Hainaut P

Cancer Genet. Cytogenet., 2008 Oct;186(1):49-53.
PMID: 18786442 DOI: 10.1016/j.cancergencyto.2008.06.004

We report on a Malaysian kindred with Li-Fraumeni syndrome. The proband was an 8-year-old girl who presented with embryonal rhabdomyosarcoma of the trunk at the age of 8 months and developed a brain recurrence at the age of 7 years, which was 5 years after remission. A younger sister later developed adrenocortical carcinoma at the age of 6 months. Their mother and maternal grandmother were diagnosed with breast cancer at the ages of 26 and 38 years, respectively. TP53 mutation detection in this family revealed a duplication of a GGCGTG motif starting at nucleotide 17579 in exon 10, resulting in an in-frame insertion of two amino acids between residues 334 and 336 in the tetramerization domain of the p53 protein. This mutation was found in the proband and her affected sister as well as her mother. In addition, the mutation was detected in two other siblings (a brother aged 3 years and a sister aged 18 months) who have not yet developed any malignancy. Sequencing of TP53 in the father and two other asymptomatic siblings revealed wild-type TP53. To our knowledge, this is a first report of a Li-Fraumeni syndrome family in Southeast Asia.

Matched MeSH terms: Soft Tissue Neoplasms/genetics
Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma - a rare vascular neoplasm with deceptive morphology and distinctive immunophenotype

Cheo FF, Sittampalam K

Malays J Pathol, 2017 Dec;39(3):305-309.
PMID: 29279595

Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma is a rare, low grade vascular (endothelial) neoplasm typically presenting as multicentric, superficial to deep nodules in extremities with a slight tendency of affecting young adult males. We report a case of pseudomyogenic hemangioendothelioma in a 15-year-old boy presenting initially with a 1 cm right thigh painless cutaneous lump. The lump was excised with the clinical impression of a sebaceous cyst. On microscopy, a poorly circumscribed, mild to moderately atypical spindle cell lesion in fascicular and storiform patterns with strikingly myoid-like eosinophilic cytoplasm was identified. The spindle cells were highlighted by pancytokeratin AE1/AE3, CD31, and ERG with retained INI-1, while being negative for MNF116, S100, CD34, EMA, desmin, SMA, caldesmon, myogenin, MyoD1, HHV-8 and CD163. Following the first diagnostic report, a positron emission tomography-computed tomography (PET-CT) scan revealed another 4 cm ill-defined nodule accompanied by a smaller adjacent 0.7 cm ipsilateral satellite nodule within the right psoas muscle that displayed similar morphology and immunophenotype as the cutaneous lump, supporting the multicentric feature of this unique entity. It is an uncommon yet increasingly recognised neoplasm of endothelial origin possessing a misleading myoid morphology and distinctive immunophenotype worth notifying.

Matched MeSH terms: Soft Tissue Neoplasms/pathology*
Fulltext Complementary and alternative medicine use amongst Malaysian orthopaedic oncology patients

Dhanoa A, Yong TL, Yeap SJ, Lee IS, Singh VA

BMC Complement Altern Med, 2014;14:404.
PMID: 25324121 DOI: 10.1186/1472-6882-14-404

Although studies have shown that a large proportion of cancer patients use CAM, no study on CAM use amongst orthopaedic oncology patients has been published. Therefore, this study aims to determine the prevalence, characteristics and factors associated with CAM use amongst orthopaedic oncology patients.

Matched MeSH terms: Soft Tissue Neoplasms/psychology; Soft Tissue Neoplasms/therapy*
Fulltext Extended pedicle rectus abdominis myocutaneous flap for thigh reconstruction

Halim AS, Zulmi W

Med J Malaysia, 2005 Mar;60(1):109-11.
PMID: 16250294

A rectus abdominis myocutaneous flap can provide a large amount of tissue for defect coverage. Rarely a flabby and redundant abdominal tissue was used as a huge extended flap. We report a case of recurrence malignant fibrous histiocytoma of the thigh which was radically resected. The resultant massive defect was success reconstructed with an extended pedicle inferiorly based rectus abdominis myocutaneous flap.

Matched MeSH terms: Soft Tissue Neoplasms/surgery*
Successful treatment of very large congenital infantile fibrosarcoma

Hamidah A, Reena M, Halim ARA, Ibrahim S, Eguchi M, Zarina AL, et al.

Pediatr Int, 2011 Oct;53(5):768-770.
PMID: 21955012 DOI: 10.1111/j.1442-200X.2011.03358.x
Matched MeSH terms: Soft Tissue Neoplasms/congenital*; Soft Tissue Neoplasms/diagnosis; Soft Tissue Neoplasms/surgery
Fulltext Blockade of c-Met-Mediated Signaling Pathways by E7050 Suppresses Growth and Promotes Apoptosis in Multidrug-Resistant Human Uterine Sarcoma Cells

Huang TT, Chen CM, Lan YW, Lin SS, Choo KB, Chong KY

Int J Mol Sci, 2022 Nov 28;23(23).
PMID: 36499211 DOI: 10.3390/ijms232314884

E7050 is a potent inhibitor of c-Met receptor tyrosine kinase and has potential for cancer therapy. However, the underlying molecular mechanism involved in the anti-cancer property of E7050 has not been fully elucidated. The main objective of this study was to investigate the anti-tumor activity of E7050 in multidrug-resistant human uterine sarcoma MES-SA/Dx5 cells in vitro and in vivo, and to define its mechanisms. Our results revealed that E7050 reduced cell viability of MES-SA/Dx5 cells, which was associated with the induction of apoptosis and S phase cell cycle arrest. Additionally, E7050 treatment significantly upregulated the expression of Bax, cleaved PARP, cleaved caspase-3, p21, p53 and cyclin D1, while it downregulated the expression of survivin and cyclin A. On the other hand, the mechanistic study demonstrated that E7050 inhibited the phosphorylation of c-Met, Src, Akt and p38 in HGF-stimulated MES-SA/Dx5 cells. Further in vivo experiments showed that treatment of athymic nude mice carrying MES-SA/Dx5 xenograft tumors with E7050 remarkably suppressed tumor growth. E7050 treatment also decreased the expression of Ki-67 and p-Met, and increased the expression of cleaved caspase-3 in MES-SA/Dx5 tumor sections. Therefore, E7050 is a promising drug that can be developed for the treatment of multidrug-resistant uterine sarcoma.

Matched MeSH terms: Soft Tissue Neoplasms*
Extraskeletal myxoid chondrosarcoma of the thigh in a child: a case report

Ibrahim ZA, Chan WH, Wong SL, Ong EJ, Narihan MZ

J Orthop Surg (Hong Kong), 2014 Dec;22(3):423-6.
PMID: 25550031

Extraskeletal myxoid chondrosarcoma (EMC) is aggressive in children. The condition in children differs to that in adults and to skeletal myxoid chondrosarcoma. We report on a 9-year-old girl with EMC in her left thigh. She underwent above-knee amputation. Five months later, a small mass was noted at the right lower lobe of the lung. The patient underwent one course of ifosfamide, carboplatin, and etoposide chemotherapy, followed by resection of the mass and 8 more courses of chemotherapy. At the 2-year follow-up, she was in remission radiologically.

Matched MeSH terms: Soft Tissue Neoplasms/pathology; Soft Tissue Neoplasms/surgery*
Fulltext Radical resection of primary leiomyosarcoma of inferior vena cava

Ikhwan Sani Mohamad, Sivabalan Nadarajan, Azim Idris, Hairol Othman, Affirul Chairil, Razman Jarmin

Malaysian Journal of Medicine and Health Sciences, 2018;14(1):61-64.
MyJurnal

Leiomyosarcoma of the Inferior Vena Cava (IVC) is a rare soft tissue tumour which accounts for only 0.5% of all soft tissue sarcomas. The patients usually presented with non specific clinical signs and often diagnosed at advanced stage. We reported a case of a 58-year-old lady who presented with right sided abdominal pain for 6 months. CT scan showed large Level 2 IVC tumour which encased the right renal vein and had no clear plane of demarcation with the caudate lobe of the liver. She underwent radical resection of the IVC tumour with the right kidney removed en bloc. Vascular reconstruction was not performed as established collateral veins present. She was nursed in Intensive Care Unit for 2 days and discharged well at day 5 post operatively. Histopathological examination results revealed leiomyosarcoma of IVC.

Matched MeSH terms: Soft Tissue Neoplasms
Fulltext Paratesticular aggressive angiomyxoma: A rare case

Ismail MI, Wong YP, Tan GH, Fam XI

Urol Ann, 2017 5 10;9(2):197-199.
PMID: 28479778 DOI: 10.4103/UA.UA_168_16

Aggressive angiomyxoma (AAM) particularly testicular origin is a rare benign mesenchymal myxoid tumor which is locally aggressive, blatant for local recurrence, and may metastasize. It occurs mostly in females of childbearing age and extremely rare in males. AMM particular testicular origin is not reported in literature yet. This is a 65-year-old man who had a right scrotal swelling. Ultrasound scrotum showed a soft tissue tumor of the right testis. The patient underwent radical right orchidectomy of which histopathologically confirmed to be a paratesticular AAM with clear resection margins. There were no signs of local recurrence or metastasis 2 years postsurgical resection.

Matched MeSH terms: Soft Tissue Neoplasms
Histopathological diagnoses in soft tissue tumours: an experience from a tertiary centre in Malaysia

Jalaludin ND, Mohd Dusa N, Hassan MR, Abd Shukor N

Malays J Pathol, 2017 Dec;39(3):209-216.
PMID: 29279582 MyJurnal

Soft tissue tumours are a group of remarkably diverse neoplasms that frequently pose significant diagnostic challenges to general pathologists. This study aimed to compare the agreement of histopathological diagnoses between general pathologists from various referral institutes and the referred soft tissue pathologist in a tertiary centre. The common discrepancies and their causes are also presented here. A retrospective study was conducted on 243 cases of potential soft tissue tumours referred to Hospital Kuala Lumpur, Malaysia over a period of 5 years. Reports by the referring pathologists and the soft tissue pathologist were compared based on tumour classification and tumour behaviour. Overall, there was moderate agreement in soft tissue tumour diagnoses in both tumour classification (weighted κ = 0.423) and tumour behavior (weighted κ = 0.548). The highest agreement of tumour classification was seen in the adipocytic tumours (21/28 cases), Ewing sarcoma (5/7 cases) and smooth-muscle tumours (3/5 cases). The highest rates of discrepancies were the so-called fibrohistiocytic tumours (7/11 cases), vascular tumours (9/15 cases) and undifferentiated/ unclassified sarcomas (19/32 cases). Full agreement for tumour behaviour was seen in 178 cases and there were 21 cases of zero agreement. Liposarcoma, alveolar soft part sarcoma and benign fibrous histiocytoma were the most frequent benign/malignant diagnostic discrepancies. The most common causes of discrepancy were wrong morphological interpretation followed by insufficient immunohistochemical stains performed. In conclusion, review of diagnosis by a pathologist specialized in soft tissue improves the quality of diagnosis in these heterogenous and rare tumours. A good panel of immunohistochemical stains with additional molecular study is crucial in the general hospital laboratories practice.

Matched MeSH terms: Soft Tissue Neoplasms/diagnosis*; Soft Tissue Neoplasms/pathology*
Mycetoma or synovial sarcoma? A case report with review of the literature

Jimenez AL, Salvo NL

J Foot Ankle Surg, 2011 Sep-Oct;50(5):569-76.
PMID: 21616683 DOI: 10.1053/j.jfas.2011.04.014

Mycetoma, also commonly referred to as Madura foot, is statistically rare in the United States. However, it is endemic to other parts of the world. It is a pseudotumor characterized by a triad of tumefaction, draining sinuses, and grains. Two types exist, with each caused by different groups of organisms that require different treatment approaches. Therefore, the exact diagnosis and culture of the organism is vital to successful treatment outcomes. Synovial sarcoma, in contrast, is a malignancy much more commonly seen in the United States. It is characterized by a well-circumscribed, often palpable, mass that is usually well delineated on magnetic resonance imaging. It has characteristic histologic and genetic features that help distinguish it from other soft tissue masses. We present a case of a soft tissue mass diagnosed in the United States. The patient had several clinical and radiographic features of synovial sarcoma but the histologic outcome was mycetoma. The case is followed by a review of the published data.

Matched MeSH terms: Soft Tissue Neoplasms/diagnosis
Fulltext Paraneoplastic Secretion of Multiple Phosphatonins From a Deep Fibrous Histiocytoma Causing Oncogenic Osteomalacia

Leow MKS, Dogra S, Ge X, Chuah KL, Liew H, Loke KSH, et al.

J Clin Endocrinol Metab, 2021 04 23;106(5):e2299-e2308.
PMID: 33462615 DOI: 10.1210/clinem/dgaa964

CONTEXT: Literature suggests that oncogenic osteomalacia is usually caused by a benign mesenchymal tumor secreting fibroblast growth factor subtype-23 (FGF-23), but the involvement of other phosphatonins has only been scarcely reported. We have previously published a seemingly typical case of oncogenic osteomalacia. Following curative neoplasm resection, we now report unique molecular characteristics and biology of this tumor.
CASE DESCRIPTION: A 25-year-old man had been diagnosed with severe oncogenic osteomalacia that gradually crippled him over 6 years. 68Ga-DOTA-TATE positron emission tomography/computed tomography scan localized the culprit tumor to his left sole, which on resection revealed a deep fibrous histiocytoma displaying a proliferation of spindle cells with storiform pattern associated with multinucleated giant cells resembling osteoclasts. Circulating FGF-23, which was elevated more than 2-fold, declined to undetectable levels 24 h after surgery. Microarray analysis revealed increased tumor gene expression of the phosphatonins FGF-23, matrix extracellular phosphoglycoprotein (MEPE) and secreted frizzled-related protein subtype 4, with elevated levels of all 3 proteins confirmed through immunoblot analysis. Differential expression of genes involved in bone formation and bone mineralization were further identified. The patient made an astonishing recovery from being wheelchair bound to fully self-ambulant 2 months postoperatively.
CONCLUSION: This report describes oncogenic osteomalacia due to a deep fibrous histiocytoma, which coincidentally has been found to induce profound muscle weakness via the overexpression of 3 phosphatonins, which resolved fully upon radical resection of the tumor. Additionally, genes involved in bone formation and bone remodeling contribute to the molecular signature of oncogenic osteomalacia.

Matched MeSH terms: Soft Tissue Neoplasms/diagnosis; Soft Tissue Neoplasms/etiology*; Soft Tissue Neoplasms/genetics; Soft Tissue Neoplasms/metabolism
Fulltext Giant epidermoid cyst of the thigh

Mohamed Haflah N, Mohd Kassim A, Hassan Shukur M

Malays Orthop J, 2011 Nov;5(3):17-9.
PMID: 25279030 MyJurnal DOI: 10.5704/MOJ.1111.001

Epidermoid cyst is a common benign cutaneous swelling frequently encountered in surgical practice. It usually presents as a painless lump frequently occurring in hairbearing areas of the body particularly the scalp, scrotum, neck, shoulder and back. Giant epidermoid cysts commonly occur in hairy areas such as the scalp. We present here the case of a rare occurrence of a giant epidermoid cyst in the less hairy area of the right upper thigh mimicking a soft tissue sarcoma. Steps are highlighted for the management of this unusual cyst.

Matched MeSH terms: Soft Tissue Neoplasms
Extensive left ventricular, pulmonary artery, and pericardial metastasis from myxoid liposarcoma 16 years after the initial detection of the primary tumour: a case report and review of the literature

Motevalli D, Tavangar SM

Malays J Pathol, 2017 Aug;39(2):201-205.
PMID: 28866706

Liposarcoma is regarded as the second most common soft tissue malignant tumour. Metastasis of liposarcoma to the heart is very rare, and to date, less than 40 cases have been reported in the literature. We report a 46 year-old male with myxoid liposarcoma of the lower extremity who developed extensive metastasis to the left ventricle, pulmonary artery, and pericardium. The patient presented with acute symptoms of dyspnea 16 years after surgical resection of the primary tumour, and his dyspnea progressed to cardio-respiratory arrest within the first few days of hospital admission.

Matched MeSH terms: Soft Tissue Neoplasms/pathology*
Fulltext Can a chronic expanding haematoma mimic a soft tissue sarcoma?

Muniandy M, Singh VA

BMJ Case Rep, 2009;2009.
PMID: 21686602 DOI: 10.1136/bcr.09.2008.0846

A 68-year-old woman presented with a gradually increasing left gluteal swelling that she had first noted 2 years previously. The results of both clinical and radiological examinations were consistent with soft tissue sarcoma, but the final diagnosis after resection was haematoma.

Matched MeSH terms: Soft Tissue Neoplasms
Fulltext A case of miliary adenocarcinoma of lung masquerading as miliary tuberculosis

Nik Azlan, N.M.

Medicine & Health, 2020;15(1):274-279.
MyJurnal

Adenokarsinoma paru-paru miliari adalah sangat jarang dan agresif. Adenokarsinoma paru-paru biasanya menunjukkan sifat ketulan pada peparu yang berkaitan dengan kerosakan paru-paru dan efusi pleura. Adenokarsinoma ini jarang jarang berpunca daripada tapak selain daripada tapak primer, tidak seperti kanser-kanser lain contohnya kanser tiroid, koriokarsinoma dan sarcoma. Kami membentangkan di sini kes seorang suri rumah berusia 50 tahun yang mengadu batuk-batuk, hilang selera makan dan sukar untuk bernafas selama 1 bulan. Perawat perubatan primer telah merawat beliau sebagai jangkitan kuman pada paru-paru. Disebabkan keadaan yang tidak pulih dan semakin melarat, beliau dibawa ke Jabatan Kecemasan. Oksigen beliau dikesan sebanyak 93% atas udara bilik. Berdasarkan sejarah pesakit dan penemuan X-ray paru-paru, rawatan untuk tuberculosis miliari telah di beri walaupun ujian Mantoux dan kahak negatif. Berikutan peningkatan tanda-tanda dan intubasi, bronkoskopi kemudian mendedahkan diagnosis adenokarsinoma paru-paru. Sebagai pelajaran, penampilan miliari radiografi tidak dikelirukan kepada tuberkulosis sahaja, kerana sebab-sebab berbahaya yang lain perlu disiasat. Bronkoskop dengan analisis histopatologi diperlukan, terutamanya jika Mantoux dan sputum adalah negatif.

Matched MeSH terms: Soft Tissue Neoplasms
Fulltext Solitary fibrous tumour of the submandibular region: a rare entity

Noor Liza Ishak, Primuharsa Putra Sabir Athar Husin, Suria Hayati Md Pauzi, Isa Mohd Rose, Mohd Razif Mohamad Yunus

Malaysian Journal of Medicine and Health Sciences, 2016;12(2):53-55.
MyJurnal

Solitary fibrous tumours of the head and neck region are
extremely rare. The clinical diagnosis is often difficult to
establish, and this lesion may be indistinguishable from other
soft tissue neoplasms. An 18-year old Chinese gentleman
presented with a painless right submandibular swelling which
was increasing in size for eight months. A computed
tomography scan showed a well-defined solid mass measuring
about 2.0 x 2.96 cm in the submandibular region. The tumour
was resected and was confined within its capsule.
Immunohistochemical staining was strongly positive for CD34,
CD 99, and vimentin and negative for desmin, smooth muscle
actin (SMA), cytokeratin, S100 and CD68. The microscopic and
immunohistochemical profile were compatible with solitary
fibrous tumour. Distinguishing solitary fibrous tumours from
various spindle neoplasms can be difficult. In view of the
resemblance, immunohistochemical staining can help
differentiate solitary fibrous tumour from spindle neoplasm.

Matched MeSH terms: Soft Tissue Neoplasms

Filters

Please provide feedback to Administrator (afdal@afpm.org.my)

External Links