When the First Impression is Not the Last: Diagnostic Challenges in Gynecologic Oncology

Uterine sarcomas.

Soft tissue sarcomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up

Objective: To find frequency of various Neoplastic and non-neoplastic lesion of the ovaries and to analyzeits clinical presentation.Method: This study was conducted from first July 2007 to 30lh June 2010 in Gynae unit B of DHQ Mardan.All the cases of ovarian Tumours which were initially recruited after ultrasonography and were laterconfirmed by open surgery were included in the study Patients record files were used for collection ofrelevant data including detailed history, examination, investigations and operative findings. All thespecimens were sent for histopathology diagnosis to histopathology section lady reading hospital and a copyof report was attached in patients record files.Result: Out of 7574 Gynanocological admission 191 cases (2.5%) of ovarian Tumours were detected. Nonneo-plastic lesion were more common 98 (51.31%) than neoplastic lesion 93 (48.69%). Among theneoplastic lesion 69.89% were benign while 30% were malignant, serous cystadenoma were the commonestbenign Tumours (35.48%) followed by mucinous cystadenoma (21.50%). Serous cyst adenocarcinoma(10.75%) was the commonest malignant Tumour followed by mucinous cystadenomcarcinoma (8.6%).Average age at presentation was 57 years for malignant ephithelial Tumours, 40 years for gonadal stromalTumours and 22.5 % for germ cell Tumours. There was significant difference in the mean age for benign andmalignant ovarian tumours(34 versus52 p =0.0001)Conclusion:Epithelial ovarian Tumours are the commonest Tumours followed by Germ cell Tumours. Agehas strong correlation with the histological type of the Tumour. Grade, histological type and age of thepatient are important prognostic factors. Ultrasound assessment of risk of malignancy favours better patientmanagement. Menstrual irregularities ,mass and pain abdomen were the commonest clinical presentations.Keywords: Ovarian Tumours, Ovarian neoplasm, Ovarian non-neoplastic lesion.

FIGO staging for uterine sarcomas

Introduction/BackgroundOvarian teratomas constitute the most common germ cell tumors (10–20%). Malignant transformation, rare at 1–3%, typically occurs in mature cystic teratomas. Moving to a different aspect of ovarian tumors, Neuroendocrine...

Endometrial stromal sarcomas are rare malignant mesenchymal uterine tumors. The expressions of different epidermal growth factor receptors such as EGFR (HER-1), HER-2, HER-3, and HER-4 have not yet been examined in these tumors. Twenty-three cases of endometrial sarcomas consisting of 20 low-grade endometrial stromal sarcomas and 3 undifferentiated endometrial sarcomas were examined immunohistochemically for EGFR (HER-1), HER-2, HER-3, and HER-4. EGFR (HER-1) was positive in 17 of 23 (74%) cases. While the three undifferentiated endometrial sarcomas were positive for EGFR, 14 of 20 (70%) low-grade endometrial stromal sarcomas showed positive reactions for EGFR. All examined cases were negative for HER-2, HER-3, and HER-4. This study is the first to show common expression of EGFR (HER-1) in endometrial stromal sarcomas. This finding may provide the basis for a new therapeutic strategy using monoclonal antibodies against EGFR (such as cetuximab) or small molecule inhibitors of EGFR (such as gefitinib) in patients with endometrial sarcomas.

BackgroundNeuroendocrine neoplasms are a heterogeneous group of cancers that develop from enterochromaffin cells of the diffuse endocrine system, with an increase in incidents over the last years. Ovarian neuroendocrine tumors (NET) are rare neoplasms, comprising 0.1% of all ovarian neoplasms and less than 5% of all neuroendocrine tumors. They may arise alone (as monodermal, specialized teratoma – ovarian carcinoid) or as a part of other ovarian lesion: cystic mature or immature teratomas. Due to the rarity and limited amount of such cases reported in the literature, there is no consensus on diagnostic and therapeutic procedures in this group of patients.Materials and MethodsThe group of 10 patients at the age of 19 to 77 years (mean 42.8 ± 17.9), diagnosed with unilateral NET within ovarian teratoma were analyzed. The histopathological type of tumor, progression free survival after surgical treatment and presence of hormonally active syndrome were assessed.Results70% (n=7) of patients was diagnosed with mature cystic teratomas containing NET component and 30% (n=3) with monodermal teratoma (strumal carcinoid). All cases of monodermal teratomas were found in women at premenopausal age. Determined Ki67 ranged from 2% to 9%. Ninety percent of lesions (n=9) stained positive for synaptophysin and chromogranin, while markers: CK20, CK7, TTF-1 and CDX2 were negative in all cases, which ruled out their metastatic nature. None of the patients presented with carcinoid syndrome. All followed-up patients remain progression-free, which confirms surgical intervention being a crucial and sufficient method of treatment.ConclusionsThe prognosis and clinical behavior of NETs associated with ovarian teratomas are good with long progression-free survival.

Use of the Thoracodorsal Artery Perforator Flap for Bronchial Reinforcement in Patients With Previous Posterolateral Thoracotomy

Post-COVID-19 Infant with a Hemizygous PRF1 Mutation and Overlapping Features between MIS-C and Hemophagocytic Lymphohistiocytosis

M.D. Chandra: None. Introduction: Neuroendocrine tumors (NETs) are rare tumors usually originating in the GI tract and lung. An ovarian neuroendocrine tumor is a rare entity, accounting for 0.5% of all carcinoid tumors and 0.1% of all ovarian. Case Presentation: A 75-year-old female presented with five days of abdominal pain. The evaluation showed normal labs and an essentially normal CT scan of the abdomen and pelvis, except for an incidental finding of a 7.8 cm right ovarian solid heterogeneously enhancing mass. MRI revealed that this mass had solid and cystic components. Later, the patient revealed an 18-pound unintentional weight loss in the past six months, with recurrent episodes of nausea, loose stools, and flushing. Evaluation for carcinoid syndrome showed an elevated 24-hour urinary 5-hydroxyindoleacetic acid [5-HIAA] at 160.8 mg, elevated serum serotonin, and chromogranin levels along with a gallium-68 dotatate scan revealing a right adnexal mass consistent with neuroendocrine malignancy. Further imaging was negative for metastasis, and upper and lower GI endoscopies were normal. She underwent tumor resection and bilateral salpingo-oophorectomy. During her exploratory laparotomy, one lesion was noted in the proximal small bowel. The ovarian tumor measured 9.1 x 9.3 X 7.4 cm, with pathology confirming its origin from the GI tract. Histology was notable for a well-differentiated neuroendocrine tumor without perineural invasion, positive for chromogranin A, immunoreactive for synaptophysin, and chromogranin. Hence, this was a metastatic small bowel neuroendocrine tumor with an isolated focus of a well-differentiated tumor in the right ovary. Four months after surgery, the patient's labs, including 24-hour 5-HIAA, were normal. The follow-up gallium scan did not show any uptake and her symptoms had resolved. Discussion: Gastrointestinal NETs, formerly called carcinoid tumors, arise from endocrine cells of the digestive tract. Well-differentiated NETs exhibit sluggish growth, and most NETs do not produce functioning hormones. Still, approximately 25% make hormones, such as serotonin, where hormone-related symptoms [diarrhea, facial flushing] are often both the source of presentation and morbidity. They can be encountered throughout the GI tract and may present symptomatically or be found incidentally. The best initial test is the 24-hour urinary excretion of 5-HIAA. Urinary and plasma serotonin and chromogranin concentrations can also be measured. The tumor must be localized and staged with the help of imaging techniques like CT or MRI. Upper and lower endoscopy must be performed for evaluation of metastatic tumors with an unknown primary site. Whole-body imaging with somatostatin receptor imaging like Octreoscan or gallium 68 dotatate is preferred, the latter being a better choice. Site-specific surgical removal helps in controlling the carcinoid syndrome symptoms. Monday, June 3, 2024

Background: Ovarian tumor is a common type of gynecological neoplasm and accounts for 15-25% of all gynecological malignancies. It is associated with high mortality and an accurate histological diagnosis is essential for management of patient.Objective: The study was performed to find out the morphological pattern, nature and age distribution of ovarian tumour in our hospital.Material and methods: It was a prospective study,conducted in the Department of Histopathology and Cytopathology, BIRDEM General Hospital, Dhaka for a period of two years from Jan 2014 to Dec 2015. This study included 186 cases of ovarian tumors sent in the Department of Pathology for histopathological evaluation. Non-neoplastic lesions and tumour-like conditions were excluded from the study. Histological diagnosis, age and laterality of ovary were recorded. Morphological pattern, nature and age distribution of ovarian neoplasms were calculated.Result: 84.95% cases of ovarian tumour were benign, 1.61% cases were borderline and 13.44% cases were malignant. ORIGINAL ARTICLES Surface epithelial tumour was the commonest type of tumour (61.83%), according to the histogenesis , followed by germ cell tumour. Benign serous tumour was the most common type of benign tumor (37.98% cases), followed by mature cystic teratoma (33.55% cases). Serous cystadenocarcinoma was the most common type of malignant tumour (36.0%), followed by endometrioid carcinoma (28.0%). Benign tumours were more frequent in all age group. The incidence of malignant ovarian tumour increased with age and was most frequent in >50 years age group. Benign tumours were commonly cystic, whereas malignant tumours were commonly solid and cystic. 11.23% cases of ovarian tumours were bilateral.Conclusion: Benign ovarian neoplasms were more common than malignant ones and benign serous tumour was the commonest type of benign neoplasm whereas serous cystdenocarcinoma was the commonest type of malignant neoplasm. The pattern and age distribution of ovarian tumour of our study were quite similar with other studies with some variation.J Bangladesh Coll Phys Surg 2018; 36(1): 5-10

Relevance. Endometrial stromal sarcomas are rare tumors that account for about 0.2% of all malignant tumors of the genital tract. It is believed that they are more common in premenopausal women. Endometrial stromal sarcomas are hormone-sensitive tumors. Because of the rarity of this tumor, there are a limited number of reports in the publications concerning the clinical control and the final result of such cases.Purpose. To study the main pathogenetic, clinical and morphological features of endometrial stromal sarcoma on the example of a clinical case.Materials and methods. The article presents a clinical case of malignant tumor of the uterus, histologically – endometrial stromal sarcoma.Results and conclusions. Diagnosis of endometrial stromal sarcoma is difficult; tumor verification requires mandatory histological examination. Difficulties in diagnosis largely determine the low early detection and poor prognosis in patients with endometrial sarcomas.

Background: Budd-Chiari syndrome (BCS) is an uncommon liver disorder characterized by the obstruction of the hepatic venous outflow tract. It is characterized by an obstruction in either the hepatic veins or the inferior vena cava. We share a case study of a female patient who initially exhibited mild symptoms, like weakness, fatigue, with a history of menorrhagia, without any overt signs of chronic liver disease. Subsequent evaluation revealed that she was actually suffering from Chronic Budd-Chiari syndrome. This condition typically presents with a classical triad of symptoms: abdominal pain, hepatomegaly, and ascites. This case highlights the diagnostic challenge posed by such disorders and the need for a detailed evaluation in a case of unexplained pancytopenia. However, atypical presentations can complicate diagnosis and management. This case report highlights an uncommon presentation of BCS, detailing its clinical course, diagnostic challenges, and management strategies. Case Report: A 24-year-old female presented with generalized weakness, fatigue, and menorrhagia. She had a history of blood transfusion a few years prior. Physical examination revealed pallor and hepatosplenomegaly, without signs of heart failure or liver disease. Laboratory workup indicated severe anemia with pancytopenia, consistent with iron deficiency anemia. Despite treatment, persistent thrombocytopenia prompted further investigation. Abdominal ultrasound confirmed hepatosplenomegaly, and a triple-phase CT scan suggested Budd-Chiari syndrome. Digital Subtraction Angiography (DSA) revealed a complete blockage of the suprahepatic inferior vena cava (IVC). Balloon venoplasty and stenting were performed to improve blood flow and reduce collateral circulation. Result: Post-procedure, the patient showed marked clinical improvement, with a decrease in hepatosplenomegaly and resolution of anemia. However, thrombocytopenia persisted, necessitating ongoing monitoring. This case underscores the importance of considering atypical presentations in the diagnosis of Budd-Chiari syndrome and highlights the efficacy of advanced interventional radiology techniques in its management. Conclusion: Budd-Chiari Syndrome can present with atypical symptoms, complicating its diagnosis. Early recognition and intervention are crucial for improving patient outcomes. Advanced imaging and interventional radiology techniques play a pivotal role in managing BCS, providing symptomatic relief and improving prognosis. Recommendations: Clinicians should maintain a high index of suspicion for BCS in patients with unexplained hepatic abnormalities and pancytopenia. A multidisciplinary approach, involving hepatologists, radiologists, and hematologists, is essential for optimal management. Regular follow-up and monitoring are recommended to manage potential complications and ensure sustained patient improvement. Keywords: Budd-Chiari Syndrome, Atypical Presentation, Thrombocytopenia, Interventional Radiology, Hepatic Vein Obstruction.

PurposeIn the present brief report, the authors summarized the data on the use of carbon ion radiotherapy in gynaecological malignancies starting from the preclinical evidence to move forward the clinically available literature and hence focusing on the possible future application directions.MethodsThis is a short report of the published studies on the role of carbon ion radiotherapy in the treatment of gynaecological malignancies.ResultsThe use of carbon ion radiotherapy in rare and radioresistant gynaecological tumors is supported by preclinical and clinical data. In particular, carbon ion radiotherapy appears to be safe and effective in the management of cervical adenocarcinomas, unresectable endometrial cancers, mucosal melanomas of the lower genital tract and vulvar adenoid cystic carcinomas. Moreover, considering the dosimetric characteristics, carbon ions are suitable for recurrent disease in the pelvis. Prospective and phase II trials on gynaecological melanomas and pelvic relapses after radiotherapy are currently ongoing. The future study directions might be the oligometastatic diseases and the oncofertility.ConclusionsMore and more growing preclinical and clinical evidence supports the use of carbon ion radiotherapy in gynaecological oncology. Strong and multidisciplinary collaborations at national and international levels are desirable to better understand the therapeutic and organizational benefits of this new technology.

Pheochromocytomas and paragangliomas are rare endocrine neoplasms derived from neural crest cells, with spermatic cord paragangliomas being exceptionally uncommon. A 42-year-old man presented with a longstanding complaint of left scrotal enlargement. Initial imaging raised suspicion of testicular carcinoma, but contrast-enhanced computed tomography and magnetic resonance imaging revealed a well-circumscribed, hypervascular mass distinct from the testis. The tumor exhibited heterogeneous T2 signal intensity, characteristic of vascular lesions, a thick capsule, and early-phase peripheral contrast enhancement with delayed homogeneous filling. A solitary fibrous tumor was initially considered as a differential diagnosis. Surgical resection confirmed the tumor's origin in the spermatic cord. Histopathology revealed small, round neoplastic cells with a delicate sinusoidal vascular network, and immunohistochemical analysis was positive for chromogranin A and synaptophysin, confirming the diagnosis of paraganglioma, with its origin traced to the spermatic cord. The surgical margins were clear, and postoperative imaging showed no metastases. At 18 months follow-up, no recurrence was detected, and biochemical markers remained normal. This case highlights the diagnostic challenges of spermatic cord paragangliomas due to their rarity and imaging resemblance to other intra-scrotal neoplasms. Although preoperative diagnosis is crucial for appropriate management, almost all of the reported cases of spermatic cord paragangliomas have been diagnosed postoperatively. New imaging techniques, including 68Ga-DOTATATE PET/CT, may change this situation. This report expands the limited literature on spermatic cord paragangliomas and underscores the importance of considering paraganglioma in the differential diagnosis of intra-scrotal masses.