Combination of Reverse Shoulder Prosthesis, Elbow Prosthesis, and Polyetheretherketone: A Modified Total Humeral Replacement for Osteosarcoma Humerus Treatment

Total humeral replacement is a complex surgery that requires many challenges to overcome such as the weight of the implant material and the shoulder function due to extensive resection of the rotator cuff. Improvements in implants material that is lighter than usual can lead to higher surgery success rates. We present a patient who was diagnosed with osteosarcoma of the right humerus. The patient received 2 cycles of MAP chemotherapy (included: doxorubicin, cisplatin, and methotrexate) before surgery. He underwent radical resection of osteosarcoma and total humerus replacement with a modified total humeral material. The purpose of this improvement was to reduce the implant’s weight and to improve postoperative recovery. Six months after the surgery, the weight-bearing ability of the patient’s shoulder within a wide range of movement has restored; the shoulder, elbow, and hand can move in a controlled way. Despite the short postoperative follow-up time, the improvement in the modified technique has brought many positive results. Total humerus replacement, which combines the reverse shoulder prosthesis, elbow prosthesis, and polyetheretherketone, is an appropriate solution for patients with osteosarcoma of the humerus instead of custom-made humerus megaprosthesis.

Abnormal Exacerbation of Moderately Differentiated Gastric Adenocarcinoma in a Patient with TAFRO Syndrome: An Impaired Tumor Immunity?

TAFRO syndrome is a relatively new disease entity first reported in 2010. We report a case of TAFRO syndrome accommodated by abnormal exacerbation of moderately differentiated gastric adenocarcinoma. The pathophysiology of TAFRO syndrome is largely unknown, but because the disease often responds to immunosuppressive therapy and also because T follicular helper (Tfh) cells are reported to be drastically decreased in TAFRO syndrome, involvement of a dysregulated immune system can be speculated. Growing evidence points toward a pivotal role of Tfh cells in tumor immunity through supporting ectopic lymphoid structures, which are recruitment sites for cells directly engaging in antitumor activity such as CD8⁺ T cells, NK cells, and macrophages. In fact, Tfh cells are reported to positively correlate with longer survival in human colorectal and breast cancer. Combined with our observations of hyperprogressive gastric cancer in the presented patient, an impaired tumor immunity is strongly indicated in TAFRO syndrome.

Sunitinib-Induced Adrenal Crisis in a Patient with Pre-Existing Immunotherapy-Related Hypopituitarism

Sunitinib is a tyrosine kinase inhibitor that is frequently used in the treatment of metastatic renal cell carcinoma (mRCC). As a multikinase inhibitor, numerous off-target side effects of this medication are widely recognized. More recently, endocrine side effects, including hypoadrenalism, are becoming more apparent. We report a case in which a 71-year-old female experienced recurrent adrenal crises when managed with sunitinib for mRCC on a background of immune-related hypopituitarism and hypoadrenalism as a result of previous treatment with immunotherapy. Clinicians should be aware of this potential toxicity when using such medications and consider further investigation in the appropriate clinical setting.

A Rare Case of a Metastatic Malignant Abrikossoff Tumor

Abrikossoff tumor, also called granular cell tumor (GCT), is a neoplasm of the soft tissues which is most commonly a solitary, painless, and benign tumor. However, 2% of Abrikossoff tumors can be malignant. We report here the case of a 75-year-old male who presented a local recurrence of Abrikossoff tumor of the left thigh. The anatomopathological analysis concluded to a malignant GCT, and the F-18 fluorodeoxyglucose positron emission tomography showed multiple lesions in the lymph nodes and bones. The potential conversion to malignancy should alert practitioners because of the extremely poor prognosis. The diagnosis of malignant granular cell tumor should be based on a bundle of clinical and histological features and not solely on histologic features because of the challenging distinction between malignant and benign tumors due to the lack of well-defined criteria for the diagnosis of malignancy. Large size and recurrence are the most important clinical features predicting malignant behavior. Patients with a history of Abrikossoff tumor should be followed closely to monitor recurrence and malignant transformation. The apparent originality of our observation – which could lie in the evolution of a GCT tumor, initially considered as benign, to a malignant form – has to be challenged regarding the issue of classifying some cases according to the classical “benign” and “malignant” dichotomy.

Bone Metastasis of Non-Small-Cell Lung Cancer Showing Pathological Complete Response to Osimertinib Monotherapy

A 70-year-old man with lung adenocarcinoma had undergone right lower lobectomy and lymph node dissection. Only 6 months later under adjuvant uracil and futraful therapy, the patient developed a solitary bone metastasis in the right 8th rib. Due to positive mutation of epidermal growth factor receptor (EGFR) exon 21 L858R in the primary cancer, the patient received osimertinib monotherapy, leading to massive calcification of the osteolytic bone metastasis with significant decrease of standard uptake value on positron emission tomography. After 12 months of osimertinib monotherapy, slight enlargement of the ground glass nodule, i.e., presumed noninvasive lung cancer, in the right upper lobe, and no further occurrence of metastatic foci made us to resect both the lung nodule and the bone metastasis. Pathological examination showed the lung nodule to be noninvasive adenocarcinoma and the bone metastasis to have no viable cancer cells. The patient was discharged on the 8th postoperative day without any complication. On developing a therapeutic strategy for advanced/recurrent EGFR mutation-positive lung adenocarcinoma, oncologists should note the possibility of pathological complete response to newly developed EGFR tyrosine kinase inhibitors including osimertinib for a presumed cure of oligometastatic lung adenocarcinoma.

Consistent Response on Challenge and Rechallenge of Liposomal Irinotecan in a Patient with Metastatic Pancreatic Adenocarcinoma Previously Treated with Gemcitabine plus Nab-Paclitaxel: A Case Report

Approximately 80% of pancreatic cancer is diagnosed at an advanced stage, due to lack of or vague symptoms when the cancer is still localized, leading to a high mortality rate. Known risk factors for developing pancreatic cancer are family history, obesity, type 2 diabetes, and alcohol and tobacco use. There has been a remarkable development in diagnosis modalities and molecular testing, but early detection is still infrequent. The majority of clinical trials have not shown significant efficacy in pancreatic cancer, and treatment strategy remains limited. Additional prognostic factors should be highlighted to obtain appropriate treatment options, including precision medicine, and improve survival outcomes. After the PRODIGE study in 2011 and the MPAC trial in 2013, a new drug (liposomal irinotecan; Onivyde ®) appeared in the strategy, especially after failure of gemcitabine-based treatment. In 2016, the NAPOLI-1 trial showed evidence of the efficacy of the liposomal irinotecan combination (liposomal irinotecan +5-fluorouracile + folinic acid); now, it is considered the standard treatment for relapsing patients. Since NAPOLI-1, real-world data have provided similar results. Herein, we report the story of a 61-year-old woman who was treated with liposomal irinotecan combination (nal-IRI/5-FU/LV) for 8 months with good surgical response, but treatment was discontinued due to economic burden. After the start of treatment (or 1? cycle of liposomal irinotecan treatment), the patient was in a better condition. The liver metastases had disappeared. The combination with liposomal irinotecan was re-administered with patient’s approval. Upon rechallenge with the liposomal irinotecan combination, she showed a partial response, and the treatment was given for 7 months. In this report, we tried to identify the prognostic factors leading to the efficacy of the liposomal irinotecan combination.

Acute Cardiac Tamponade Secondary to Cardiac Vascular-Type Pleomorphic Leiomyosarcoma: Case Report

Soft tissue sarcomas represent <1% of all neoplasms. Leiomyosarcomas comprise only 5–7% of cases, and only 2% of these are vascular. Vascular leiomyosarcomas are extremely rare and represent only 0.001% of all neoplasms, the venous type being up to 5 times more frequent. Arterial leiomyosarcomas most frequently affect the great vessels, being fatal in most cases. In the reported cases of arterial leiomyosarcomas, the most frequently affected site is the pulmonary artery. We present the clinical case of 2 patients (a 42-year-old woman and a 36-year-old man) with a diagnosis of arterial pleomorphic leiomyosarcoma that conditioned cardiac tamponade as the initial manifestation. As it is an exceptionally rare neoplasm and with few cases reported in the literature, it is important to identify and describe this pathology which, due to the impossibility of offering surgical treatment, represents a therapeutic challenge.

Bortezomib-Induced Perimyocarditis in a Multiple Myeloma Patient: A Case Report

Bortezomib (BTZ) is a proteasome inhibitor used in the treatment of multiple myeloma (MM) and other hematological malignancies. Although carfilzomib, a second-generation proteasome inhibitor, is most strongly associated with cardiotoxicity, BTZ has been associated with several cardiovascular complications including congestive heart failure, arrhythmias, and rarely myocarditis. Here, we report the first case of a BTZ-induced perimyocarditis. The patient was a 40-year-old woman with recently diagnosed MM who was admitted to the hospital with syncope at the start of her second cycle of induction therapy with BTZ, lenalidomide, and dexamethasone. She had a witnessed syncopal event in the emergency room with the telemetry showing sustained ventricular tachycardia. Laboratory workup showed elevated N-terminal pro B-type natriuretic peptide and normal troponin I. Transthoracic echocardiogram (TTE) showed a low ejection fraction of 40% with global hypokinesis of the left ventricle and trace pericardial effusion. Cardiac magnetic resonance imaging with gadolinium was consistent with acute myocarditis. The patient had recurrent pleuritic chest pain, and a repeat TTE showed worsening pericardial effusion consistent with pericarditis. Endomyocardial biopsy was done which showed nonspecific myocyte hypertrophy and foci of fibrosis, but was negative for giant cell myocarditis, hemochromatosis, and amyloidosis. Extensive infectious disease workup ruled out known infectious causes for perimyocarditis. Given the close timing between BTZ treatment (5 subcutaneous doses with a cumulative dose of 6.5 mg/m²), the absence of other iatrogenic or infectious causes, and probable or likely association with BTZ as assessed by the validated causality assessment scoring tools, it was concluded that the acute perimyocarditis was secondary to BTZ exposure. Here, we report the first case of BTZ-induced perimyocarditis and discuss the incidence and pathophysiology of BTZ-cardiovascular toxicity.

Occult Disseminated Metastatic Breast Carcinoma Presenting as Acquired Thrombotic Thrombocytopenic Purpura

Cancer-related microangiopathic hemolytic anemia (MAHA) is a rare and life-threatening condition. We present a patient who had been treated for invasive lobular breast carcinoma in clinical remission with fever and hemolytic anemia. The peripheral blood film showed MAHA and thrombocytopenia, and a functional deficiency of ADAMTS13 activity of 23% consistent with acquired thrombotic thrombocytopenic purpura. Bone marrow aspirate and trephine biopsy confirmed metastatic carcinoma. Further evaluation revealed the involvement of multiple bone sites without recurrence of the primary tumor. The patient received a daily plasma exchange with cryosupernatant and was pulsed with corticosteroids. MAHA related to breast cancer appears to be a rare occurrence.

Successful Response to Cyclophosphamide, Vincristine, and Dacarbazine Chemotherapy in a Patient with Metastatic Carotid Body Paraganglioma

Carotid body paraganglioma is a rare neuroendocrine tumor presenting with low-grade histological and clinical features. However, the tumor has the potential to produce distant metastasis, and due to its rarity, little information is available regarding chemotherapy for such metastatic lesions. Here, we report a case of carotid body paraganglioma with development of pulmonary and bone metastases 10 years after radical surgery for the primary lesion in the neck. The lesions showed a good response to cyclophosphamide, vincristine, and dacarbazine chemotherapy. A beneficial therapeutic outcome by chemotherapy is extremely rare in patients with metastatic carotid body paraganglioma.