Primary cardiac atrial sarcomas. Report of two histologically different cases and review of the literature.

Primary cardiac sarcomas are extremely uncommon. We report two patients with primary cardiac atrial sarcomas: a case report of a 34-year old woman with intimal sarcoma of the left atrium and a case report of a 30-year old man with synovial sarcoma of the right atrium. Clinicopathological and differential diagnosis with a discussion regarding the role of molecular studies is presented.

Cisplatin Monotherapy as a Treatment Option for Patients with HER-2 Negative Breast Cancer Experiencing Hepatic Visceral Crisis or Impending Visceral Crisis.

INTRODUCTION: Hepatic visceral crisis (VC), characterized by a rapid total bilirubin increase with disease progression, poses a life-threatening risk in advanced breast cancer (ABC). International consensus guidelines define VC and touch on impending VC (IVC). Limited data exist on systemic treatments for hepatic VC/IVC. This study explores the safety and efficacy of cisplatin monotherapy in patients with Human Epidermal Growth Factor Receptor 2- negative breast cancer (BC) and hepatic IVC/VC. METHODS: In this retrospective single-center cohort study data of patients treated with cisplatin monotherapy (60-80 mg/m2, every 3-4 weeks) between 2016 and 2023 at a reference Cancer Centre in Southern Poland were analyzed. RESULTS: 33 female patients (24/33 hormonal-positive) with the mean age 53.84 years were included. Participants progressed on median 2 prior palliative systemic treatment lines. In 10/23 patients hepatic VC and in 23/33 IVC (rapid, symptomatic liver progression; extensive liver involvement; alanine or aspartate aminotransferase > 2 × normal limit; significant increases in lactate dehydrogenase, alkaline phosphatase, or gamma-glutamyl transferase) were identified. Median progression-free survival was 1.87 months and median overall survival 2.67 months. 33% of the patients presented stable disease or partial response. Eight patients experienced adverse events grade ≥ 3: in five the dose of cisplatin was reduced; two stopped the treatment. CONCLUSION: Due to the hepatotoxicity of BC-active drugs, specific recommendations for systemic treatment are scarce. Our study explored cisplatin's potential use, finding it to be a viable option in patients with performance status 0 or 1 experiencing hepatic IVC/VC, irrespective of liver function parameters and other factors.

Non-metastatic primary neuroendocrine neoplasms of the breast: a reference cancer center's experience of a heterogenous entity.

Background: Primary neuroendocrine neoplasms of the breast (Br-NENs) are rare. The classification has been updated in recent years making interpretation of the data published challenging. It is unclear whether neuroendocrine differentiation is associated with poorer prognosis and what treatment approaches should be applied. Methods: The database for breast cancer patients treated between 2009 and 2022 at the Maria Sklodowska-Curie National Research Institute of Oncology Branch Krakow was explored to search for Br-NENs. Patients' medical and pathological data were collected and analyzed. Results: We included 22 females with Br-NEN without metastases at the time of diagnosis. The median age was 64 years (range: 28-88), Of the cases, 18 were hormone receptor positive, all were HER-2 negative, the median Ki67 was 27% (10-100%). The median tumor size at the time of diagnosis was 29.5mm (7-75mm), 9 patients were N-positive. DCIS was present in 5 cases. Only one case was negative for chromogranin and synaptophysin staining, but data were missing for 4 cases. Nine patients received adjuvant chemotherapy, mainly based on anthracyclines and taxanes, while 16 received adjuvant hormonal therapy and 15 received postoperative radiotherapy. Radical surgery was performed in all patients, but two underwent suboptimal tumorectomy. One patient had local recurrence, three experienced metastatic disease, all involving the lungs, but these patients are still alive. The median follow-up was 96 months (8-153). Two patients died, with a follow up time of no recurrence >4 years. Our results were compared to twelve case series collecting clinical data on Br-NENs, with median patient number of 10.5 (range: 3-142). Conclusion: Br-NENs represent a heterogenous group of diseases, lacking data from prospective studies or clinical trials. There are no established treatment standards tailored for Br-NENs. Our patients' cohort exhibited a favorable prognosis, potentially attributed to lower tumor stage and Ki67 index compared to other reported case series. We suggest that radical surgery and postoperative radiotherapy be administered akin to standard treatment for breast cancer of no special type. ESMO also advocates for this approach in systemic treatment, although we recommend considering platinum-based chemotherapy for patients with poorly differentiated Br-NENs exhibiting high Ki67.

Stereotactic Radiotherapy in Combination with Immunotherapy in Treatment of Advanced Recurrent Squamous Cell Carcinoma of the Larynx.

Squamous cell carcinoma (SCC) of the larynx in advanced stages is a challenging malignancy to treat with a high recurrence and death rate. An individualized approach to treatment is crucial in such patients. We present a 58-year-old male patient with SCC of the larynx in the T3N0M0 stage who was treated with concurrent radiochemotherapy. A total of 17 months after the radical treatment, the patient underwent a laryngectomy due to recurrence. A total of 11 months after the operation, local failure was diagnosed. In the next order, the patient received six cycles of palliative chemotherapy according to cisplatin 100 mg/m2 and 5-fluorouracil 1000 mg/m2. After three months, due to progression, Nivolumab-based immunotherapy was administered, ensuring disease stabilization. After the 56th cycle of Nivolumab, another progression was documented. The addition of stereotactic radiotherapy (18 Gy in three fractions) to immunotherapy led to significant regression of the disease and enabled the continuation of Nivolumab to the 70th cycle. The presented case demonstrates the usefulness of the combination of stereotactic radiotherapy with immunotherapy in prolonging the local control.

Stereotactic Radiotherapy Boost in Treatment of Persistent Periocular Sebaceous Carcinoma after Surgery.

Sebaceous carcinoma is a rare malignancy that should be treated with surgical resection. Nonetheless, a dynamic and aggressive course of the disease may disqualify a patient from this treatment. Applying radiotherapy with the escalation dose using a stereotactic boost is worthy of consideration as a radical treatment. In this paper, we present the case study of a young patient with a tumor localized in the periocular area. The patient was treated with operation two times without a satisfactory effect. Conventional radiotherapy, 60 Gy in 30 fractions, combined with chemotherapy based on cisplatin 40 mg/m2 and the addition of a stereotactic radiosurgery boost were administered. The tolerance of this treatment was acceptable. During the 2-year follow-up, local and distant recurrences were not diagnosed. The presented case shows the usefulness of an individualized approach in the radical treatment of sebaceous carcinoma with the use of the stereotactic radiotherapy boost. This is a subsequent example of the implementation of the boost in head and neck carcinoma, which yields a positive result.

Escalating a Biological Dose of Radiation in the Target Volume Applying Stereotactic Radiosurgery in Patients with Head and Neck Region Tumours.

Background: The treatment of head and neck tumours is a complicated process usually involving surgery, radiation therapy, and systemic treatment. Despite the multidisciplinary approach, treatment outcomes are still unsatisfactory, especially considering malignant tumours such as squamous cell carcinoma or sarcoma, where the frequency of recurrence has reached 50% of cases. The implementation of modern and precise methods of radiotherapy, such as a radiosurgery boost, may allow for the escalation of the biologically effective dose in the gross tumour volume and improve the results of treatment. Methods: The administration of a stereotactic radiotherapy boost can be done in two ways: an upfront boost followed by conventional radio(chemo)therapy or a direct boost after conventional radio(chemo)therapy. The boost dose depends on the primary or nodal tumour volume and localization regarding the organs at risk. It falls within the range of 10-18 Gy. Discussion: The collection of detailed data on the response of the disease to the radiosurgery boost combined with conventional radiotherapy as well as an assessment of early and late toxicities will contribute crucial information to the prospective modification of fractionated radiotherapy. In the case of beneficial findings, the stereotactic radiosurgery boost in the course of radio(chemo)therapy in patients with head and neck tumours will be able to replace traditional techniques of radiation, and radical schemes of treatment will be possible for future development.

High-dose stereotactic radiotherapy boost in the treatment of squamous cell carcinoma of the head and neck region.

BACKGROUND: Surgical resection with adjuvant concurrent radiochemotherapy is the standard of care for stage III-IV oral cavity cancer. In some cases, the dynamic course of the disease is out of the prepared schedule of treatment. In that event, a stereotactic radiosurgery boost might be the only chance for disease control. CASE PRESENTATION: Here, we present a case study of a patient with oral cancer who underwent surgery. During adjuvant radiotherapy, a metastatic cervical lymph node was diagnosed based on fine-needle aspiration biopsy. To increase the total dose to the metastatic tumor, a stereotactic radiosurgery boost of 1 × 18 Gy was performed two days after the last fraction of conventional radiotherapy. The early and late tolerance of this treatment were positive. During the 18-month follow-up, locoregional recurrence was not detected. The patient died due to secondary malignancy. CONCLUSIONS: This paper shows that a stereotactic radiosurgery boost added to adjuvant conventional radiotherapy is an effective approach permitting the maintenance of good local control in well-selected patients.

Successful Treatment of Adenoid Cystic Carcinoma with the Application of a High-Dose Stereotactic Body Radiotherapy Boost.

BACKGROUND: Adenoid cystic carcinoma (ACC) should be treated with a surgical procedure. Unfortunately, in some cases, such procedures are impossible to perform. In that event, radiotherapy can be used as a form of radical treatment, although ACC is established as a radio- and chemoresistant tumour. Therefore, unconventional fractionated radiotherapy needs to be considered. CASE PRESENTATION: Here, we present a case study of a patient with an unresectable tumour of the choanae and nasopharynx treated with a stereotactic radiotherapy boost in combination with conventional radiotherapy. We achieved complete clinical regression after application of a 1 × 18 Gy boost followed by conventional radiotherapy at 50 Gy in 25 fractions. The early and late tolerance of this treatment were positive. During the 2-year follow-up, local and distant recurrence were not detected. CONCLUSIONS: This case represents an individualized, modern and safe approach to unresectable ACC. This is one of the first cases to show the use of a combination of stereotactic and conventional radiotherapy in radical, conservative cancer treatment.