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Background: Gestational Trophoblastic Neoplasia (GTN) is a disease of the reproductive age group with an incidence rate of <1% among all tumors involving the female reproductive tract. It occurs because of aberrant fertilization. Patients are diagnosed early because of aggravated symptoms during pregnancy. Moreover, patients also bleed from the tumor sites, which leads to early presentation. A cure rate of 100% can be achieved with adequate treatment. Main body: In this literature review, the authors have brought to attention the risk factors, classification, and various treatment options in GTN patients according to their stratification as per the WHO scoring system. Patients are categorized into low and high risk based on the FIGO scoring system. Patients with low risk are treated with single-agent methotrexate or actinomycin-D. Despite the superiority of actinomycin-D in terms of efficacy, methotrexate remains the first choice of therapy in low-risk patients due to its better toxicity profile. Multi-agent chemotherapy with etoposide, methotrexate, actinomycin-D, cyclophosphamide and vincristine (EMA-CO) leads to complete remission in 93% of high-risk GTN patients. Around 40% of patients with incomplete responses are salvaged with platinum-based multi-agent chemotherapy. Isolated chemo-resistant clones can be salvaged with surgical interventions. Conclusion: The mortality in patients with GTN has significantly reduced over time. With adequate multi-disciplinary support, patients with GTN can ultimately be cured and can spend every day healthy reproductive life. [ABSTRACT FROM AUTHOR]

Objective: To determine the recurrence-free survival (RFS) in patients with squamous cell carcinoma of oral cavity (OSCC) Methodology: It was a longitudinal study conducted on oncology patients who were diagnosed as having squamous cell carcinoma of the oral cavity and treated in our tertiary care hospital in the department of medical oncology. Patients were followed for the evaluation of disease recurrence. The Chi-square test was used to see the association between outcomes and clinicopathological features of the tumor. Results: Of 97 patients, 85(87.62%) patients had no disease, however, 12(12.37%) patients had residual/recurrent disease in the first post-treatment follow-up scans performed three months after the completion of curative treatment. Subsequent follow-up scans showed disease recurrence in 87(89.69%) patients, out of which 52(59.77%) had loco-regional disease recurrence while 35(40.22%) patients had combined loco-regional and systemic disease recurrence with lung(n=18) being the most common site of distant metastasis. The mean RFS was 15 months (IQR= 8, 26). Patients with lymph vascular invasion (LVI), perineural invasion (PNI), positive margin, and extranodal extension(ENE) had a significantly higher risk of progressive disease after primary treatment, on follow up scans. The relationships between treatment outcomes and LVI, PNI, positive margin and ENE were statistically significant (p<0.05). Conclusion: Our study shows that there is a higher frequency of recurrence in patients with OSCC among patients with extra nodal extension, lymph vascular and perineural-invasion, and positive margins. Clinical trials are required to identify other poor prognostic factors and the need of developing a prognostic model for risk-stratification of such patients. [ABSTRACT FROM AUTHOR]

Background and aim: Docetaxel, oxaliplatin, leucovorin and 5-fluorouracil (FLOT) may improve overall survival (OS) in patients with locally advanced gastric and gastroesophageal cancer. Our study aims to determine the pathological response in these patients with the FLOT chemotherapy in the Neoadjuvant setting. This is the first study conducted in our country. Methods: We conducted a retrospective cross-sectional study from March 2018 to December 2020. After ethical review committee approval, all patients who fulfilled the inclusion criteria and received treatment at our tertiary care center were included in the study. SPSS version 22 was used for data analysis. Frequencies and percentages were calculated for categorical. Values were presented as mean ± standard deviation (SD) for continuous variables. The chi-square test was used to determine the difference between categorical variables. A p-value of ≤0.05 was considered the level of significance. Kaplan- Meier curves were used to calculate survival analysis. Results: Out of 41, 35 patients with locally advanced resectable gastric or gastroesophageal adenocarcinoma were included in our study analysis. The entire cohort had a male predominance, with a mean age of 59. All patients received neoadjuvant FLOT. Pathological treatment response achieved was 77%, of which 66% had partial and 11% had complete response. There is a significant association of pathological response with age, gender, stage, grade, co-morbid and number of chemotherapy cycles received (p-value =<0.05). The OS was 80% with the mean OS was 2.6 years (31 months). Conclusion: Our study shows comparable response rates to other studies conducted internationally. Our findings confirm that FLOT is an effective and well-tolerated perioperative regimen with reasonable response rates in the Pakistani population. A more extensive longitudinal study would ensure these preliminary results in the local patient population. [ABSTRACT FROM AUTHOR]

Primary malignant melanoma of the head and neck region is an exceptionally unique neoplasm that accounts for 1 % of all mucosal melanomas diagnosed worldwide. Most patients are either symptomless or have vague symptoms. In this report, we describe the case of a young female, who presented at a tertiary care institute in Pakistan, with a history of recurrent ipsilateral mucosal neoplasm arising in the nasal cavity. The patient was treated with surgical resection twice and was subsequently found to have widespread metastatic lymph nodes on workup. Malignant mucosal melanoma seldom originates from the nasal cavity. Surgical resection is the best chance of cure for localized nasal melanomas whereas for metastatic disease, systemic therapy with either chemotherapy or biologic agents is the mainstay of management. Approximately 5 % of the cases of mucosal melanoma have metastatic disease at presentation. This report highlights the presentation, clinical characteristics, management, and prognosis of non-cutaneous melanoma, arising within the head and neck region. • The nasal cavity is an uncommon site of origin for mucosal melanoma. • Although asymptomatic in the majority of the cases, symptoms may include nasal congestion, nasal swelling, or obstruction. • In this report, we present a case of recurrent nasal melanoma with metastatic nodal involvement. • The prognosis is poor and, palliative treatment with systemic agents is the standard in advanced stages. [ABSTRACT FROM AUTHOR]

Acinic cell carcinoma (AciCC) is a rare entity in which high-grade transformation (HGT), formerly dedifferentiation, is uncommon. This case report presents a rare case of AciCC, with rapid transformation to metastatic high-grade dedifferentiated pathology after initial curative treatment. A 58-year-old woman presented in the medical oncology clinic with a progressive swelling on the right side of her face. Magnetic resonance imaging revealed a 5 × 5 cm lobulated parotid gland lesion, and fine needle aspiration biopsy was consistent with carcinoma. After informed consent, a Modified-Blair incision was given as a standard approach to the right preauricular area under general anaesthesia, and a right superficial parotidectomy with the removal of the tumor and selective lymph node dissection was performed. Histopathology of the resected mass was reported as parotid AciCC. She was given adjuvant radiation therapy. A repeat PET CT scan ten weeks after the completion of her adjuvant radiation treatment showed local disease recurrence as well as multiple pulmonary deposits. A repeat biopsy was reported as DOG-1 positive dedifferentiated (high-grade) acinic cell carcinoma, and she was offered platinum-based palliative systemic chemotherapy. Parotid acinic cell carcinomas with high-grade transformation are rare. This case highlights its critical diagnostic markers, curative and palliative management and long-term follow-up. The transformation of parotid AciCC to high-grade, dedifferentiated pathology is unusual and belligerent. Hence, these tumors need intense treatment with a multimodality approach. Close follow-ups with history and physical examination, along with periodic imaging, should be considered for these patients. • Parotid acinic cell carcinoma is a rare entity with the potential of meteoric transformation to high-grade pathology • A multimodality approach, including surgery, and radiation therapy with or without chemotherapy, should be considered. • Platinum-based systemic chemotherapy and PDL-1 targeted immunotherapy may help palliate symptoms in the metastatic setting. • Next-generation sequencing tests may help to identify gene alteration that can be therapy-target. [ABSTRACT FROM AUTHOR]

Paraneoplastic syndromes are complex clinical manifestations that occur because of the underlying malignancy in which the malignant cells produce hormones, cytokines, peptides or antibodies that causes symptoms and may affect multiple organ systems. These paraneoplastic conditions may be associated with different solid and hematological malignancies. Multiple Myeloma (MM) accounts for 10–15 % of hematological malignancies and 1–2 % of all malignancies. It is associated with some atypical clinical and laboratory paraneoplastic manifestations. Although there is a low incidence of these paraneoplastic, significant knowledge of these manifestations may assist in making a differential diagnosis in cases of doubt. The clinical presentation may vary and be evident even before or after the diagnosis of malignancy. These include vascular, neurological, dermatological, physiological, and other atypical conditions. Furthermore, these rare paraneoplastic manifestations need more valid, relevant scientific information, as most information about these conditions is derived from case reports. After the literature search, we have reported the paraneoplastic manifestations associated with multiple myeloma, published in the English literature, and the cognate management in this review article. To our knowledge, this is the first review article discussing various paraneoplastic manifestations of multiple myeloma. [ABSTRACT FROM AUTHOR]

Lung cancer is one of the most common solid malignancies. Tissue biopsy is the standard method for accurately diagnosing lung and many other malignancies over decades. However, molecular profiling of tumors leads to establishing a new horizon in the field of precision medicine, which has now entered the mainstream in clinical practice. In this context, a minimally invasive complementary method has been proposed as a liquid biopsy (LB) which is a blood-based test that is gaining popularity as it provides the opportunity to test genotypes in a unique, less invasive manner. Circulating tumor cells (CTC) captivating the Circulating-tumor DNA (Ct-DNA) are often present in the blood of lung cancer patients and are the fundamental concept behind LB. There are multiple clinical uses of Ct-DNA, including its role in prognostic and therapeutic purposes. The treatment of lung cancer has drastically evolved over time. Therefore, this review article mainly focuses on the current literature on circulating tumor DNA and its clinical implications and future goals in non-small cell lung cancer. [ABSTRACT FROM AUTHOR]

Primary cardiac sarcoma is a rare malignancy with a poor prognosis because of diagnostic delay, therapeutic difficulties, and high metastatic potential. The therapeutic approach includes surgery, chemotherapy, and radiation therapy, alone or in combination. However, there is a lack of evidence to guide the treatment. We present a case of primary cardiac sarcoma. Our patient was presented in the department of emergency medicine (ED) in our institute with shortness of breath on exertion associated with orthopnea. Based on the history and cardiovascular examination, he underwent an echocardiogram, which revealed a sizeable echogenic density in the right ventricular outflow tract. He underwent surgical resection of the cardiac mass via median sternotomy and total cardiopulmonary bypass approach. The patient was eventually diagnosed with primary cardiac sarcoma, confirmed by tissue biopsy after surgical intervention. Through this report, we highlight the rarity of primary cardiac sarcomas, the importance of multidisciplinary tumor board (MDT) discussion and provide evidence of surgical excision being the treatment of choice, followed by systemic chemotherapy in selected cases. Cardiac sarcoma is a rare but highly malignant tumor with a poor prognosis. However, early diagnosis and surgical resection of a primary cardiac sarcoma can significantly increase the patient's survival and quality of life. Therefore, physicians should keep a high suspicion of a patient with clinical features suggestive of cardiac sarcoma, and echocardiography should be the diagnostic modality of choice in such patients. • Primary Cardiac Sarcoma is a rare malignancy with a poor prognosis because of its highly malignant potential. • Evidence to guide treatment should be based on multidisciplinary tumor board (MDT) discussion. • Surgical excision followed by chemotherapy may improve survival and quality of life. [ABSTRACT FROM AUTHOR]

Myoepithelial carcinomas are a diverse group of tumors exhibiting myoepithelial differentiation. There have been increasing reports of extra-salivary sites of origin for myoepithelial carcinomas such as soft tissues, bone and visceral areas. Due to this entity's rarity, definite diagnostic and treatment parameters are somewhat limited. We present the case of a myoepithelial carcinoma arising from the abdominal wall, a rare site of origin of an uncommon tumor. A 37-year-old gentleman presented to our institution in Oct 2018 with a recurrent abdominal mass for which he underwent wide local excision after completing the workup, which included systemic scans and relevant blood investigations. The histopathology report was consistent with malignant abdominal myoepithelial carcinoma. However, subsequent follow-up scans in May 2019 showed disease progression with the appearance of multiple lung metastases. After a detailed discussion, he was started on Pazopanib 800 mg orally once a day, on which he remained stable till May 2022. It was then when he experienced clinical disease progression confirmed on systemic scans, so he was offered palliative systemic chemotherapy. Abdominal malignant myoepithelial carcinomas are an infrequent entity. However, this case highlights its critical diagnostic markers and primary and recurrent abdominal myoepithelial carcinoma management. Abdominal myoepithelial carcinomas, although rare, are also under-recognized. Thus, keeping an index of high suspicion for these tumors and being armed with knowledge regarding the heterogeneity of its features would lead to better diagnostic awareness and documentation, paving the way for better evidence-based treatments. • Extra-salivary myoepithelial carcinoma is an extremely rare entity, may present as an abdominal mass. • In contrast to their salivary-gland counterparts, most of them are malignant to begin with. • Limited data is available for their management, hence such patients should be enrolled in clinical trials whenever available. [ABSTRACT FROM AUTHOR]

The neoplasms of the testis are sporadic tumors among men. Furthermore, the rarest subset of these is neoplasms belonging to the stromal tumors of the sex cord. Leydig cell tumors are the most common form among the testicular stromal tumors. In our case, the underlying risk factor associated with the development of Leydig cell tumors was cryptorchidism. While mostly unilateral, cases of bilateral cryptorchidism may be present and have been rarely reported. We report a 36-year-old gentleman who presented to us with the inability to carry off intercourse without difficulty attaining erection on stimulation for the past two years. He had a history of left undescended testis since birth, for which he underwent left orchidopexy 20 years ago. An ultrasound of the pelvis showed an oval hypoechoic-shaped heterogeneous mass in the right mid-inguinal canal. Relevant blood investigations showed a deranged hormonal profile. He then underwent an uneventful right radical orchiectomy, histopathology of which was consistent with Leydig cell tumor. LCT with a history of bilateral cryptorchidism has rarely been reported. This case highlights its clinical presentation, management, and further follow-up in such patients. Bilateral congenital cryptorchidism may be associated with Leydig cell tumor years later in life hence long-term follow-up is required for these patients. The clinical presentation of these tumors may vary among individuals. Any change in physical appearance, hormonal assay, and imaging studies should promptly be followed for possible surgical resection and close monitoring. • Bilateral congenital cryptorchidism may be associated with LCT, years later in life. • The clinical presentation of these tumors may vary among individuals. • Such patients need periodic monitoring of hormonal profiles and imaging studies. [ABSTRACT FROM AUTHOR]