Intermediate Malignant Potential Research Articles

Pulmonary Inflammatory Myofibroblastic Tumour: a case report

Inflammatory myofibroblastic tumour (IMT) is a rare mesenchymal tumour that occurs predominantly in children and young adults. Etiology remains unclear. But based on the frequent detection of chromosomic alterations, especially near the Anaplastic Lymphoma Kinase gene, IMT is now considered to be a true neoplasm. In addition, the possible aggressive behaviour, and the ability to metastasize, suggest at least an intermediate malignant potential. Surgery remains the treatment of choice, but the use of chemotherapy, non-steroidal anti-inflammatory drugs (NSAID), immunotherapy and targeted therapy are reported. We describe a case of a pulmonary IMT in a six-year-old boy with an incidental finding of a lesion in the right upper lobe. A video-assisted thoracoscopic right upper lobectomy with lymph nodes resection was performed. Microscopic examination confirmed the diagnosis of IMT with the nodule showing spindle cells in a background of plasma cells. Anaplastic Lymphoma Kinase (ALK) immunohistochemical expression was negative. ALK inhibitors show promising results in ALK-expressing IMT. Nevertheless, primary surgery is the treatment of choice with a good overall survival rate in case of complete resection. But prolonged follow up is mandatory, as cases of delayed metastasis are described. Keywords: Inflammatory myofibroblastic tumour, paediatrics, thoracic surgery, Anaplastic Lymphoma Kinase, histopathology

Spectrum of blue nevus-like lesions, including blue nevus, pigmented epithelioid melanocytoma & animal-type melanoma

Blue nevus-like lesions constitute a category of melanocytic lesions clinically identified by their blue coloration. Histologically, they exhibit two primary features: a dermal location and intense pigmentation. The latest World Health Organization (WHO) classification categorizes blue melanocytic lesions into benign entities (dermal melanocytoses, blue nevus, and deep penetrating nevus), melanocytic tumors with low to intermediate malignant potential (pigmented epithelioid melanocytoma, PEM), and malignant lesions (blue nevus-like melanoma and melanoma arising in blue nevus).Clinically, blue nevi are enduring and stable lesions, displaying a structureless blue pigmentation both clinically and dermatoscopically, with a straightforward histologic diagnosis. Conversely, lesions with recent onset and/or rapid growth are more commonly associated with diagnoses falling within the intermediate part of the spectrum or with melanoma. These lesions often present with a blue color along with additional features such as black blotches, irregular vessels, and irregular pigmented globules. They typically emerge de novo without recognizable precursors, they pose significant challenges for patient management.Melanoma on a blue nevus is an exceedingly rare entity with only a few cases described to date. Histologically, differentiating between lesions with intermediate malignant potential and melanoma is always challenging, necessitating a comprehensive evaluation of all morphologic findings of the lesion.

Rare inflammatory myofibroblastic lung tumor with multi-organ metastasis: A case report

Inflammatory myofibroblastic tumors (IMTs) are extremely rare neoplasms, accounting for 0.04–0.7% of all tumors. Data on their clinical presentation, pathogenesis, and biological behavior are scarce. They can occur at any age, involving equally males and females. IMTs have been described as benign neoplasms with intermediate malignant potential, as local recurrence and distant metastasis may occur. IMTs are characterized by a solid growth type, and a multifocal type is uncommon. Definitive surgery is the treatment of choice. We describe a clinical case of a malignant IMT with multi-organ metastasis 6 years after definitive surgery on the primary tumor. There is no standard of care in such a clinical situation, especially given the absence of mutations in the primary and metastatic tumors.

Unravelling the Enigma of Inflammatory Myofibroblastic Tumours: A Cross-sectional Study from a Regional Cancer Centre, Karnataka, India

Introduction: Inflammatory Myofibroblastic Tumour (IMT) is an unusual neoplasm with intermediate malignant potential, which rarely metastasises. IMT occurs in various anatomical sites, and the initial descriptions were primarily centered on its manifestation in the lungs. Although the age range is broad, IMT is most common in the first three decades of life, with a slight female predominance. Recent data and Anaplastic Lymphoma Kinase (ALK) gene aberrations confirm a neoplastic process for these lesions. Aim: To study the clinicopathological features of IMTs in a regional cancer centre in South India. Materials and Methods: A cross-sectional study included 17 cases of IMT over six years, from May 2014 to May 2020, at Memorial Kidwai Memorial Institute of Oncology (KMIO), Bengaluru, Karnataka, India. The clinicopathologic and immunophenotypic features were analysed on needle biopsies and resected specimens. Correlation between the expression of ALK-1 and histological patterns, mimickers, metastasis, and prognosis were described. Results were analysed using Microsoft Excel 2019 and Medcalc calculator. Results: Seventeen cases of IMTs were included in this study, out of which two were in the lung, and the rest were extrapulmonary. Sixteen cases were unifocal on presentation; however, one IMT of extremity showed evidence of secondaries on bone scan. The mean age at presentation was 33.47 years, and the maleto-female ratio was 1:1.12. Among the seventeen cases, sites are as follows: one each in the retroperitoneum, liver, soft-tissue (extremity), two each in the breast, lung, female genital tract, urinary bladder, and three each in the head and neck region (maxilla, trachea, and alveolus) and mesentery. Microscopically, IMTs showed various histological patterns. Immunostaining for ALK-1 was positive in seven out of seventeen cases, and the rest were diagnosed by excluding closer mimickers. Conclusion: The present study provides crucial insights into the clinicopathological features of IMTs. Immunohistochemistry (IHC) is an essential diagnostic tool that helps accurately differentiate IMT from its mimickers by identifying ALK protein expression. This distinction is essential for guiding targeted therapy in recurrent or metastatic cases.

Abdominal inflammatory myofibroblastic tumour: Clinicopathological and molecular analysis of 20 cases, highlighting potential therapeutic targets.

Inflammatory myofibroblastic tumour (IMT) is a rare mesenchymal neoplasm of intermediate malignant potential, occurring at any age and at multiple sites. Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is an aggressive subtype of IMT, typically involving the abdomen. Most IMTs harbour kinase gene fusions, especially involving ALK and ROS1, but 20-30% of IMTs show no detectable translocations. The aim of this study is to further delineate clinicopathological and molecular characteristics of abdominal IMT and discover potential new therapeutic targets. In 20 IMTs, including four EIMS, RNA fusion analysis was performed, followed by multiplex DNA analysis if no ALK or ROS1 fusion was detected. Fourteen IMTs (70.0%) had an ALK translocation and the fusion partner was identified in 11, including a RRBP1::ALK fusion, not previously described in classical (non-EIMS) IMT. RANBP2::ALK fusion was demonstrated in all EIMS. One IMT had a ROS1 fusion. In all ALK/ROS1 translocation-negative IMTs mutations or fusions - as yet unreported in primary IMT - were found in genes related to the receptor tyrosine kinase (RTK)/PI3K/AKT pathway. Three of four patients with EIMS died of disease [mean survival 8 months (4-15 months)], whereas only one of 14 classical IMT patients succumbed to disease [mean follow-up time 52 months (2-204 months); P < 0.01]. This study shows the wide clinical spectrum of abdominal IMTs and affirms the poor prognosis of EIMS, raising discussion about its status as IMT subtype. Furthermore, the newly detected alterations of the RTK/PI3K/AKT pathway expand the molecular landscape of IMTs and provide potential therapeutic targets.

Cytopathologic features of epithelioid hemangioendothelioma including touch imprints for rapid on-site evaluation.

Epithelioid hemangioendothelioma (EHE) is a vascular tumor of intermediate malignant potential, which presents as infiltrative lesions involving multiple organs. We reviewed our institutional experience with the cytologic diagnosis of this neoplasm including the performance of rapid on-site evaluation (ROSE). From our institutional database, we identified 29 cytology specimens, obtained between 2012 and 2020, from 21 patients with biopsy confirmation of EHE. ROSE and final diagnosis were compared. All cytology slides were reviewed, and selected cytologic features were recorded. The cohort included 29 specimens comprising 17 (59%) from liver, 6 (21%) from lung, 2 (7%) from lymph node, and 4 (14%) from other sites. At ROSE, 8/27 (30%) were reported inadequate, yet on review, all cases contained scattered cells typical of EHE in the touch imprint air-dried slides including two cases reported with a final diagnosis of non-diagnostic. All cases contained epithelioid and plasmacytoid cells with ovoid nuclei, fine chromatin, delicate (or biphasic) cytoplasm, and scattered cells with delicate, elongated cytoplasmic tails. The majority 26/29 (90%) of cases had multi-nucleated and multi-lobated nuclei. Intracytoplasmic lumens/blister cells were in 17/29 (59%), and a subset had erythrocytes therein (4/29, 14%). Metachromatic fibromyxoid or fibrotic stroma fragments were commonly seen (23/29, 79%). Mitoses and necrosis were absent in all cases. Of 11 tested cases, WWTR1::CAMTA1 and YAP1::TFE3 fusions were detected in nine and two cases, respectively. EHE has distinctive cytologic features which are often under-recognized during ROSE.

Patient with composite haemangioendothelioma containing angiosarcoma-like areas in the setting of congenital lymphoedema mimicking Stewart-Treves syndrome: a case report

BackgroundComposite haemangioendothelioma is a rare vascular neoplasm with indolent to intermediate malignant potential. Diagnosis of this disease relays on histopathological identification of at least two different morphologically distinctive vascular components in proper clinical settings. Exceedingly rare cases of this neoplasm can exhibit areas resembling high-grade angiosarcoma, which does not change the biological behaviour. Such lesions tend to occur in the setting of chronic lymphoedema and thus, can mimic Stewart-Treves syndrome, which has a much worse clinical outcome and prognosis.Case presentationWe present a case of 49 years old male suffering from chronic lymphoedema of the left lower extremity who had developed a composite haemangioendothelioma with high grade angiosarcoma-like areas mimicking the Stewart-Treves syndrome. Given the multifocality of the disease, the only potentially curable surgical treatment would be hemipelvectomy, which was refused by the patient. The patient has been followed-up, with no signs of local progression of the remaining disease, nor a distant spread outside the involved extremity for two years.ConclusionsComposite haemangioendothelioma represents a rare malignant vascular tumour, with significantly more favourable biological behaviour than angiosarcoma, even in cases where angiosarcoma-like areas are present. For that reason, composite haemangioendothelioma can be easily misdiagnosed as true angiosarcoma. The rarity of this disease unfortunately hampers the development of clinical practice guidelines and the implementation of treatment recommendations. Most of the patients with localized tumour are treated by wide surgical resection, without neo- or adjuvant radiotherapy or chemotherapy. However, in the case of this diagnosis, the watch-and-wait approach is better than mutilating procedure, highlighting the necessity of establishing of the correct diagnosis.

Congenital Atrophic Dermatofibrosarcoma Protuberans: A Case Report and Review of the Literature

Dermatofibrosarcoma protuberans (DFSP) is a rare mesenchymal tumor of intermediate malignant potential. The neoplasm is locally aggressive with a high rate of recurrence. It typically presents in adults. Atrophic congenital DFSP is extremely rare. The few reported cases have presented as a morphea-like plaque that persists for years, before progressing into a nodular form. To our knowledge, congenital atrophic DFSP has been only reported fourteen times, and of those, only nine were confirmed by molecular studies. Herein we report a congenital case of atrophic DFSP, which initially presented as a bruise-like atrophic plaque on the dorsal forearm, initially mistaken for child abuse. The clinical appearance, histopathology, and molecular features of this rare form of DFSP are reviewed. Our case highlights the importance of early detection and adequate sampling of congenital DFSP; early treatment allows for treating small lesions without large, disfiguring, and potentially disabling excisions.

A Rare Case of Incidentally Diagnosed Pulmonary Inflammatory Myofibroblastic Tumour with Dramatic Response to Crizotinib in a Postpartum Woman.

Inflammatory myofibroblastic tumours are rare neoplasms which most commonly affect children and young adults. With an intermediate malignant potential, they are typically detected in the abdomen, lung, mediastinum, head and neck, gastrointestinal tract, and genitourinary tract. We describe the case of a 33-year-old postpartum woman incidentally diagnosed with a pulmonary inflammatory myofibroblastic tumour following complaints of poorly controlled hypertension a week after caesarean section. She was ALK-negative and received an ALK inhibitor with complete resolution of the lesion. A ROS1-TFG fusion confirmed the diagnosis of an inflammatory myofibroblastic tumour after CT-guided fine needle aspiration. This case highlights an uncommon presentation posing a diagnostic and therapeutic challenge and the potential treatment option of crizotinib. Inflammatory myofibroblastic tumour (IMT) is a rarely reported neoplasm arising in the abdominal soft tissues, the lung, mediastinum, head, neck, gastrointestinal tract, and genitourinary tract, with intermediate malignant potential.IMT is definitively diagnosed only after histological examination following surgical biopsy, based on immunohistochemical markers and the molecular characteristics of the tumour, but small biopsies may have a role in a large lesion.IMT did not cause any complications during gestation.Therapeutic approaches include surgical resection and chemotherapy, including with crizotinib, an ALK tyrosine kinase inhibitor.

DERMATOFIBROSARCOMA PROTRUBERANS- A RARE CASE REPORT

Dermatobrosarcoma protruberans (DFSP) is a rare mesenchymal sarcoma with low to intermediate grade malignant potential that typically involves the dermis with an incidence rate of 0.8 to 4.5 cases per million persons per year. Typical presentation is an asymptomatic, indolent, exophytic protruberant tumour with a high local recurrance rate seen on the trunk. We report a typical case of DFSP with local recurrence on the left lower back in a 32-year old female which was managed with wide local excision with margins free of tumour inltration conrmed by histopathology. This case is reported for its rarity

Inflammatory myofibroblastic tumour of an unusual site in an infant

Inflammatory myofibroblastic tumors are rare mesenchymal tumors of intermediate malignant potential, usually seen in the first or second decades of life. They have a high local recurrence rate but rarely metastasize. The involvement of paranasal sinuses or the nasal cavity is relatively uncommon, especially in infants. In children, there is a predilection for the abdominal cavity and it is rare in infants. Complete surgical excision is the treatment of choice. We report a case of a 10-month-old girl with an inflammatory myofibroblastic tumor of the left nasal cavity and sinuses who was initially treated with chemotherapy in view of the complicated surgical process and later underwent near-total resection as she was not responding to chemotherapy. Knowledge of such tumors among physicians and ENT surgeons will improve the prognosis, as completely excised tumors have a very low recurrence rate and adjuvant therapy may not be needed.

Clinical and histopathological analysis of 141 dermatofibrosarcoma protuberans in Korea: A comparative study according to trauma.

Dermatofibrosarcoma protuberans (DFSP) is a rare fibrohistiocytic tumour of unknown pathogenesis with intermediate malignant potential. Although trauma has been hypothesized as a predisposing factor for DFSP development, clinicopathological characteristics of trauma-related DFSP have not been investigated. This study investigated the differences between trauma-associated DFSP and trauma-unrelated DFSP. Patients histopathologically diagnosed with DFSP from January 2000 to December 2019 at the Dermatology Department were included. Clinical, histopathological, prognostic features and trauma history were analysed. We recruited 141 patients with DFSP (mean age, 36.1 years; male: female, 1:1.01). Recurrence and systemic metastasis were observed in 15.6% and 2.8% of patients, respectively. Older patients were likely to develop DFSP at the trauma sites more frequently on the face and lower legs. The active-growing lesions were more frequently associated with trauma-related DFSP. Multivariable logistic regression revealed that age at diagnosis (OR: 1.031; 95% CI: 1.004-1.059; p=0.024) and tumour growth (OR: 3.336; 95% CI: 1.162-9.578, p=0.025) were significantly associated with trauma-related DFSPs. The age at diagnosis, lesion location and tumour growth were associated with DFSPs in this study. Analysis of DFSP with trauma history provides a deeper understanding of long-term trauma effects on sarcoma development.

Inflammatory Myofibroblastic Tumor of the Placenta With Subsequent Successful Pregnancy and Benign Hysterectomy: A Case Report With 59-Month Follow-up.

Inflammatory myofibroblastic tumors (IMT) are rare neoplasms of intermediate malignant potential which have been described in the gynecologic tract, predominantly in the myometrial wall, but also in association with the placenta. Like those in other organs, IMT of the placenta are characterized by molecular abnormalities, most commonly anaplastic lymphoma kinase gene rearrangements, and are often positive for anaplastic lymphoma kinase immunohistochemically. Although the clinical behavior of placental IMTs has so far proven benign, a successful intrauterine pregnancy with subsequent negative hysterectomy following a placental IMT has not been documented. Herein is presented a case of a 27-yr-old noted to have a 2cm IMT of the extraplacental membranes at delivery, after which the patient received no further treatment. After 56mo, the patient experienced a subsequent normal delivery in a pregnancy complicated by gestational diabetes. No longer desiring fertility, the patient elected to have a hysterectomy to confirm the absence of IMT at 59mo and the uterus was unremarkable. This case provides insight into possible outcomes for patients with a rare tumor who may desire future fertility and may otherwise be advised to undergo hysterectomy in the setting of an unclear clinical course.

Epidemiology, clinical features and outcomes of ganglioneuroblastoma: An analysis of the National Cancer Database.

e22013 Background: Ganglioneuroblastoma (GNB) is a rare tumor of the sympathetic nervous system composed of both immature neuroblasts and mature ganglion cells. It has an intermediate malignant potential that lies between a low grade ganglioneuroma and a high-grade neuroblastoma and has the capability to transform into a neuroblastoma. While more information is known regarding pediatric neuroblastoma, little is known regarding the epidemiology and clinical features of GNB. To our knowledge, this is the first analysis of ganglioneuroblastoma using the National Cancer Database (NCDB) and potentially the largest and most comprehensive studies of this subtype of tumor to date. Methods: Using the National Cancer Database (NCDB), 273 patients diagnosed with ganglioneuroblastoma (ICD-0 9490/3) from 2004-2018 were identified. Kaplan-Meir analysis was used to estimate survival and log-rank tests were used to compare survival between stage groups. Results: The median age of diagnosis for patients with ganglioneuroblastoma was found to be 4 years. Girls were affected more commonly than boys, with a male to female ratio of 1.6/1. A majority of patients were white (77.7%) and African American (13.6%), followed by minor representations from multiple other races. The most common primary site of ganglioneuroblastoma was the thorax (40.5%), followed by the abdomen (35.7%). A minority of patients (14%) had distant metastases at diagnosis. At the time of disease presentation, 56% of patients were classified as stage I, 9.2% as stage II, 18.4% as stage III and 16.3% as stage IV. Stages I, II, and III conferred an excellent prognosis with a 99.2% survival rate as compared to patients with stage IV disease who had a survival rate of 65.2% (p &lt; 0.001). Nearly all patients underwent surgery of the primary site, with the most common approach being local excision. Around one quarter of patients received chemotherapy while very few patients required radiation or immunotherapy. Conclusions: A number of unique findings help to distinguish ganglioneuroblastoma from the more common variant neuroblastoma. GNB seems to affect slightly older children (median age of 4 years) compared to a median age of 22 months in neuroblastoma. Interestingly, GNB was found to occur significantly more frequently in girls. On the contrary, neuroblastoma has been reported to be slightly more common in males than females. Additionally, the majority of ganglioneuroblastomas arise in the thorax. This is in comparison to neuroblastomas, of which the large majority arise in the abdomen, specifically in the adrenal glands. Stage IV GNB carries a significantly worse prognosis than stages I-III, with a survival rate of 62.5% which is similar to stage IV neuroblastoma.

A Giant Solitary Fibrous Tumour of the Pleura

A solitary fibrous tumour of the pleura (SFTP) is a rare pathology, frequently benign in nature, and is usually diagnosed incidentally on imaging. We herein describe the case of a previously fit and well, 35-year-old Caucasian lady, who presented to us with a history of progressively worsening shortness of breath. Her chest X-ray showed a near-complete opacification of the right hemithorax, with displacement of the mediastinum towards the left. This study was supplemented by a computed tomography (CT), which demonstrated a well-circumscribed, non-homogenous mass, occupying the entirety of the right hemithorax. A large, smooth, encapsulated tumour was surgically resected via a posterolateral thoracotomy, measuring approximately 23.1 cm x 21.0 cm x 11.5 cm and weighing 3640 grams. Histopathology confirmed the diagnosis of a benign SFTP with an intermediate malignant potential. At six months, a follow-up CT scan demonstrated no evidence of disease recurrence.

Histopathological Types of Papillary Thyroid Carcinoma: Clinicopathologic Study

BACKGROUND: Papillary thyroid carcinoma (PTC) is the most common type of thyroid carcinoma that represents a set of characteristic of nuclear features in which the diagnosis is depend. AIM: The study aimed to review different variants of PTC which has different malignant potential in correlation with many prognostic and clinical factors in Iraq. PATIENTS AND METHODS: Paraffin blocks of 227 cases of PTC were selected, subtyped, and grouped according to the malignant potential. The prognostic and clinical factors were studied. RESULTS: The mean age was (39.29 ± 12.17) years, with female predominance (86.3%), where (55.5%) of them below 40 years, conventional variant was most common (40.1%), followed by papillary microcarcinoma variant, 197 cases (86.8%) were classified as low to intermediate malignant potential, and 30 cases (13.2%) were classified as high malignant potential. On comparison, among the tumor stages, tumor focality, native capsular invasion, lymph nodes involvement, presence or absence of vascular invasion, extrathyroid extension, and distant metastasis, there is no significant correlation between them, whereas with the tumor size, there is significant correlation between them (p =&lt; 0.05). CONCLUSION: Conventional type followed by micropapillary type is most common. The mean age in Iraqi patients is lower than other countries for PTC. There is no significant association regarding prognostic factors between low to intermediate malignant potential variants and high malignant potential variants with exception of tumor diameter.

Giant cell angioblastoma

Giant cell angioblastoma is an extremely rare tumor of vascular origin, described at the end of the 20th century. It belongs to tumors with intermediate malignant potential and is characterized by locally infiltrative growth. The tumor doesn’t have any clear distinctive clinical characteristics. The diagnosis is established on the basis of histological examination. Two main treatment options for this pathology are discussed in the literature: radical removal of the tumor and therapy with low doses of interferon alpha. As a rule, this is a combination treatment. This article describes our own clinical case. The patient’s parents gave their consent to the use of their child’s data, including photographs, for research purposes and in publications. Interest is in the rarity of the disease and the features of the clinical characteristics of this case, specifically the extremely unfavorable localization in the oropharynx region and, accordingly, the impossibility of carrying out not only a radical removal of the tumor, but also its resection. The high probability of developing irreversible neurological complications in this age group associated with interferon alpha therapy questioned the possibility of its use. For the first time in this histological variant of a vascular tumor, chemotherapy was applied, including metronomic therapy with cyclophosphamide and vinblastine in combination with a liposomal form of doxorubicin. After 8 courses of chemotherapy, a complete clinical response was obtained with the restoration of the patency of the respiratory and digestive tracts. The observation period at the time writing of this article was 36 months.

Dermatofibrosarcoma Protuberans of the Back – A Case Report

Dermatofibrosarcoma protuberans (DFSP) is a rare tumour of the soft tissue tumor involving the dermis, subcutaneous tissue, and in few cases, muscle and fascia. The tumour typically presents as a slowly growing, firm plaque or nodular on the trunk and extremities of young adults. The etiology of dermatofibrosarcoma protuberans has been attributed to a chromosomal translocation t (17;22)(q22;q13) that results in a fusion protein promoting tumour growth through the overproduction of platelet-derived growth factor (PDGF). Diagnosis is made via skin biopsy. Treatment options include complete surgical excision by performing conventional surgery with wide margins (&gt;3 cm) or Mohs micrographic surgery. These lesions are locally aggressive with high rate of recurrence following surgery but the prognosis is excellent when it is treated effectively. These tumours have intermediate malignant potential.

Inflammatory myofibroblastic tumor: molecular landscape, targeted therapeutics, and remaining challenges

Inflammatory myofibroblastic tumor (IMT) is a rare mesenchymal tumor of intermediate malignant potential that predominantly affects children, adolescents and young adults. IMT has a predilection for the lung, abdomen, pelvis, and retroperitoneum, however, can affect any part of the body. IMT is typically localized, and multifocal or metastatic disease is uncommon. Complete surgical resection is the treatment of choice when feasible. There is no established standard of care for unresectable and advanced IMT. Approximately half of IMTs harbor anaplastic lymphoma kinase (ALK) gene rearrangements, and fusions involving ROS1, PDGFRβ, RET and NTRK have also been described. Given the molecular landscape of IMT, management of these tumors has evolved to include tyrosine kinase inhibitors and novel targeted therapeutics. This review highlights the molecular characteristics, evolution of targeted therapies and the remaining challenges in the management of IMT.

A Five-Year Review of Soft Tissue Tumors with Intermediate Malignant Potential and Soft Tissue Sarcomas in a Tertiary Hospital: University of the Philippines – Philippine General Hospital

Objective. Describe the epidemiology of Soft Tissue Tumors with Intermediate Malignant Potential (STTI) and Soft Tissue Sarcomas (STS) diagnosed in Philippine General Hospital, Department of Laboratories, Section of Surgical Pathology, from years 2014 to 2018. Methodology. We utilized a descriptive, retrospective, cross-sectional study design and involved all newly-diagnosed cases of STTI and STS that fit the specified set of inclusion and exclusion criteria. Results. Out of 1896 cases of probable STTI and STS on initial screening, 680 cases (36%) were included in the study. Of the 1216 excluded cases, 815 (43%) needed ancillary diagnostic workup for definitive classification. Sarcoma, Not Otherwise Specified (n=149; 21.9%; 95% CI [18.80, 25.02]) was the most common diagnosis, followed by gastrointestinal stromal tumor (n=91; 13.4%; 95% CI [10.82, 15.94]) and leiomyosarcoma (n=62; 9.1%; 95% CI [6.95%, 11.28%]). Median age was 47 years, with a slight female predominance (n=371; 55%; 0.83 male to female ratio). The extremities (n=244, 36%) were the most frequent site. Conclusion. The significant amount of cases excluded in the study may account for the differences of distribution. Despite the increased immunohistochemistry tests available, there is still an apparent inaccessibility to ancillary diagnostic methods that are necessary in the diagnosis of STTI and STS.