Erythroderma: An unusual manifestation of imatinib - A rare case report.

Imatinib is a tyrosine kinase inhibitor that selectively inhibits several protein tyrosine kinases which is central to the pathogenesis of human cancer. It forms the first-line treatment for chronic myeloid leukemia (CML) and gastrointestinal stromal tumors. Usually, the drug is well-tolerated with relatively few side effects. Adverse effects most commonly associated with imatinib include mild-to-moderate edema, nausea and vomiting, diarrhea, muscle cramps, and cutaneous reactions. Other side effects such as the elevation of hepatic transaminase and myelosuppression occur less frequently and resolve with interruption of imatinib therapy. Skin rash is one of the most common adverse effects of imatinib incidence of which range from 7% to 88.9%. Exfoliative dermatitis, i.e., erythroderma has been very rarely reported with this drug. We here report a rare case of erythroderma in a patient with CML on imatinib 400 mg/day therapy within 3 months of starting the treatment.

Clinico-pathological correlates and survival outcomes in 214 resected ampullary adenocarcinomas - are outcomes different in intestinal and pancreatobiliary subtypes with adjuvant gemcitabine?

BACKGROUND: Evidence suggests that intestinal type (IT) and pancreatobiliary (PB) subtypes of ampullary adenocarcinoma (AC) may have different outcomes. The current study evaluated differences in outcomes between these subtypes and the benefit of adjuvant chemotherapy (AT). METHODS: A prospectively maintained database of patients who underwent upfront resection for AC from January 2012 to March 2016 was conducted. A dedicated pathologist reported differentiation between IT and PB subtypes. RESULTS: 214 patients were included for analysis: 105 PB subtype and 109 IT subtype. With a median follow up of 46.3 months, estimated 4 year overall survival (OS) was 65.8%. In patients with stage II-III disease, lymph-node ratio (LNR) < 0.2 [Not reached (NR) vs. 30.72 months; p = 0.002], absence of perineural invasion (PNI) (NR vs. 31.61 months; p = 0.032) and AT (gemcitabine - 96.1%) (NR vs. 22.28 months) were prognostic for superior OS. There was no difference in OS between IT and PB subtypes, but both subtypes with stage II-III disease benefitted from AT statistically as compared to observation (IT: NR vs. 28.62 months; PB: 18.46 months vs. 58.09 months; p < 0.001). CONCLUSIONS: AC-IT and AC-PB did not have a different OS when treated with resection and adjuvant gemcitabine, though adjuvant therapy benefitted both subtypes individually.

Hemangiopericytoma of supraglottis: A rare case report and review of literature.

In 1942, Stout and Murray first used the term hemangiopericytoma to describe a tumor which is distinguished histologically from other types of vascular neoplasm characterized by proliferation of pericytes. It is a rare neoplasm that was originally described as a vascular tumor derived from the pericytes. They account for 2%-3% of all soft tissue sarcomas in humans and they occur mainly in the musculoskeletal system. About 15%-30% of all hemangiopericytomas occur in the head and neck region. Hemangiopericytoma of supraglottis is very rare neoplasm with only nine cases reported in literature and ours is the tenth case overall and first case in pediatric age group. Herein, we are presenting an extremely rare case report of hemangiopericytoma of supraglottis in a 6-year-old male child who presented with stridor followed by which tracheostomy was done. Later, the patient was treated initially with radiotherapy followed by surgery, i.e., laryngectomy in view of residual disease postcurative radiotherapy. Hence, hemangiopericytoma is a very rare tumor overall and can present in pediatric age group and can be one most important differential diagnosis because many patients in this age group, stridor most commonly occurs due to the infectious causes such as influenza virus and diphtheria-induced croup, i.e., laryngotracheobronchitis.

Low prevalence of deficient mismatch repair (dMMR) protein in locally advanced rectal cancers (LARC) and treatment outcomes.

BACKGROUND: The available evidence in locally advanced rectal cancer (LARC) suggests a low prevalence of deficient mismatch repair (dMMR) protein status, approximating 1-3%. METHODS: Patients with LARC who were offered long course chemoradiation (LCRT), as per institution protocol during the period of 1st January 2014 to 31st December 2015 at Tata Memorial Hospital (TMH) in Mumbai were evaluated for outcomes and assessment of MMR status. RESULTS: A total of 419 patients were evaluated for LARC in TMH, of whom 354 were treated with LCRT. Of these 354 patients, 296 were assessable for MMR status based on tissue adequacy for testing. Three patients (1.01%) has dMMR status, while the remaining 293 patients had proficient MMR status. A total of 240 patients (67.8%) underwent curative intent resections. With a median follow-up of 32 months, estimated 3-year recurrence free survival (RFS) and overall survival (OS) for the resected group was 63.5% and 85.2%, respectively, while 3-year event free survival and OS for the unresected cohort was 15.2% and 15.8%, respectively. Signet ring histology, higher ypT stage, involved margin status post resection, and delays (>1 week) in LCRT were associated with inferior OS on multivariate analysis. CONCLUSIONS: In a large LARC cohort, a majority of tumors had proficient MMR status, suggesting that MSI as a biomarker may have limited applicability in the management of rectal cancers. Signet ring histology, CRM involvement post resection, higher ypT stage and interruptions in LCRT predicted for inferior OS.

Methotrexate-Induced Toxic Epidermal Necrolysis: A Rare Case Report and Review of Literature.

Acute lymphoblastic leukemia (ALL) is the most common malignancy in pediatric patients, and it is characterized by the presence of malignant lymphoblasts within the bone marrow and peripheral blood. The treatment of ALL involves induction, consolidation, reinduction, and maintenance therapy. Consolidation therapy in ALL-Berlin-Frankfurt-Münster 90 protocol involves the use of high-dose methotrexate (HDMTX, 5 g/m2) over 24 h as continuous infusion. The adverse effects due to HDMTX include renal dysfunction in 2%-12% patients, which can lead to increased systemic MTX exposure, leading to further myelosuppression, mucositis, hepatotoxicity, skin toxicity, and, in severe cases, multiorgan failure. Dermatologic toxicity due to MTX includes morbilliform drug rash, photoreactivation, photoenhancement, and skin hyperpigmentation. Stevens-Johnson syndrome and toxic epidermal necrolysis (TEN) are rare and possibly fatal reaction which can occur with MTX. Here, we describe a patient with B-cell ALL who developed TEN after administration of HDMTX.

A study of the use of peripherally inserted central catheters in cancer patients: A single-center experience.

Effective and reliable venous access is one of the cornerstones of modern medical therapy in oncology. The focus of this prospective observational research is to study the various indications of a peripherally inserted central catheter (PICC) in different solid and hematological malignancies and the various complications and outcomes in the pediatric and adult cancer patients. This study was conducted in a prospective observational study design and collected data of patients with a diagnosis of any cancer, at a tertiary care oncology hospital in Ahmadabad, Gujarat, India, during a 2-year period. The PICC was inserted in 352 patients and most commonly used in hematological conditions (n = 295, 83.8%), followed by solid malignancies 57 (16.2%). In the hematological malignancy group, acute myeloid leukemia (48.01%) was the most common indication, and in the solid malignancies group, osteosarcoma (n = 9, 2.55%) was the most common indication for PICC insertion. PICCs were inserted most commonly in the left side of the venous system in 70.7% cases. The complications in the PICC study group included infections (12.5%), thrombosis (4.82%), catheter blockage (4.82%), arrhythmias (4%), premature catheter removal (3%), bleeding (2.55%), and pneumothorax (2.55%). The median days of the PICC use in situ were 152 days. To conclude from our study, PICCs are most commonly indicated in malignancies that are requiring long-term chemotherapy, such as hematological malignancy, especially acute myeloid leukemia, and solid malignancies, usually osteosarcoma, and these catheters are associated with complications such as infection, thrombosis, catheter blockage, arrhythmia, bleeding, and pneumothorax. The most disturbing aspect of the treatment of a cancer patient is multiple painful venipunctures made for administration of cytotoxic agents, antibiotics, blood products, and nutritional supplements. From this study, we can infer that PICC lines can be used for various malignancies that require long-term chemotherapy.

Cutaneous Metastasis Leading to the Diagnosis of Carcinoma of the Prostate: A Rare Case Report and Review of Literature.

Prostate adenocarcinoma is a common urologic malignant neoplasm in man. Distant cutaneous metastases (CMs) of prostate carcinoma are extremely rare with a reported incidence of 0.36% and usually they occur late. Clinically, cutaneous metastasis of prostate carcinoma can mimic other skin conditions such as cellulitis, sebaceous cyst, zosteriform lesions, telangectasias, and more, resulting in a poor recognition. Few cases of true cutaneous metastatic prostate carcinoma exist in the literature. We present a case, where the first sign of carcinoma of the prostate was CM over the anterior abdominal wall. Radiological and histopathological confirmation pointed to a diagnosis of carcinoma of the prostate. The patient was treated with orchidectomy and was started on bicalutamide. After 1 month of bicalutamide therapy there was subjective decrease in the size of the metastasis. A high index of suspicion is required while evaluating the different differential diagnoses of this entity particularly in elderly patients.

Rare occurrence of hand-foot syndrome due to paclitaxel: A rare case report.

Hand-foot syndrome (HFS) is a relatively frequent adverse reaction to certain anticancer drugs. HFS is a type of dermatitis which has been most commonly described with 5-fluorouracil and capecitabine. However, HFS with paclitaxel is rare and has been reported sparingly in the literature. A 52-year-old male patient with recurrent carcinoma of the buccal mucosa was started on palliative chemotherapy regimen, injection paclitaxel (175 mg/m2) in combination with injection carboplatin. On post-chemotherapy day 13, the patient started developing pain, dysesthesia followed by bullae formation, and desquamation over palms and soles. Clinically, the patient had Grade 3 HFS characterized by symmetrical, tender skin lesions over the dorsal aspect of palms, and soles with desquamation necessitating interruption of treatment. Therefore, this case has been presented to be cognizant with this rare form of side effect with one of the most commonly used drug in oncology.

Tamoxifen Induced Pancreatitis: An Unusual Complication of Commonly used Drug.

Tamoxifen is a selective oestrogen receptor modulator used for the treatment of oestrogen/progesterone receptor positive breast cancer. It possess antagonistic or agonistic activity depending on the tissue location i.e., antagonistic action on breast but agonist action on endometrium and bones. The side effects of tamoxifen include hot flushes, gynaecologic symptoms (vaginal dryness, vaginal discharge), depression, forgetfulness, sleep alterations, weight gain, alteration of lipoprotein metabolism, thromboembolic disorder. Tamoxifen, like oestrogens, increases the plasma level of triglycerides and liver secretion of Very Low Density Lipoprotein (VLDL). Moreover, it inhibits the key enzymes of triglyceride metabolism. However, there are few cases of severe tamoxifen induced hypertriglyceridemia and pancreatitis. Hypertriglyceridemia is one of the risk factor for acute pancreatitis. Here we present a case of tamoxifen-induced hypertriglyceridemia and acute pancreatitis in a 50-year-old female without any comorbidity. She was treated with supportive antibiotics and supportive therapy. About one week after discharge, patient was started on letrozole 2.5 mg once a day. Clinicians must be aware of this rare side effect of tamoxifen, so baseline and periodic testing of triglyceride level must be done to avoid such complications.

Ifosfamide-induced Encephalopathy Precipitated by Aprepitant: A Rarely Manifested Side Effect of Drug Interaction.

Central nervous system (CNS) toxicity has been reported in approximately 10%-30% of patients receiving intravenous infusions of ifosfamide. Encephalopathy is a rare but serious CNS adverse reaction in these patients, and although usually transient and reversible, may cause persistent neurological dysfunction or death. Clinical features range from fatigue and confusion to coma and death. Ifosfamide forms backbone of various treatment regimens including curative treatment and palliative chemotherapy regimen. Precipitation of ifosfamide-induced encephalopathy (IIE) by aprepitant has been reported in the literature rarely. Ifosfamide is moderately emetogenic; hence, aprepitant is used to prevent emesis induced by ifosfamide. We here report a case where a patient of recurrent B-cell Philadelphia-negative acute lymphoblastic lymphoma was given aprepitant to prevent ifosfamide-induced emesis. After 24 h of ifosfamide infusion, the patient developed symptoms of encephalopathy, i.e., headache, vomiting, and one episode of seizure which was followed by disoriented behavior. After doing all routine investigations and neuroimaging, the diagnosis of IIE was kept on clinical grounds, and after looking for the various factors, we came across injection fosaprepitant as the precipitating factor. On the clinical grounds, the patient was treated with hydration and injection methylene blue for above complaints, and the patient recovered without any residual deficit within 48-72 h. Hence, in the presence of causative agent, i.e., ifosfamide and precipitating agent injection fosaprepitant with negative imaging and normal laboratory parameters as well as the early and good response to methylene blue, the diagnosis of IIE precipitated by aprepitant was confirmed.

Primary Mediastinal Synovial Sarcoma Presenting as Superior Vena Cava Syndrome: A Rare Case Report and Review of the Literature.

Primary mediastinal sarcomas are aggressive tumors with a very rare incidence. This report describes the case of a 35-year-old male patient who presented with acute symptoms of dyspnoea, facial puffiness, voice-hoarseness, and engorged neck veins. With the clinical picture consistent with the superior vena cava (SVC) syndrome, the patient was investigated with computed tomography of the chest. This revealed a large soft tissue density mass lesion compressing the SVC along with other critical superior mediastinal structures. Histopathological evaluation of the mass revealed features consistent with a soft tissue sarcoma and positive staining was observed for vimentin and S-100. Cytogenetic analysis by fluorescent in situ hybridisation (FISH) demonstrated the t(X:18) translocation. Thus diagnosis was established as primary mediastinal synovial sarcoma. Patient was treated with three cycles of neoadjuvant chemotherapy, to which there was a partial response as per the RECIST criteria. Surgical excision of the mediastinal mass was performed, and further postoperative treatment with adjuvant chemoradiotherapy was provided. Patient currently is free of disease. This is to the best of our knowledge the first report in the world literature of a successfully treated case of "primary mediastinal sarcomas presenting as SVC syndrome." Patient is under regular surveillance at our clinic and remains free of recurrence one year after treatment completion.

Paraneoplastic encephalitis as a first evidence of recurrent neuroblastoma: A rare case entity.

Prevalence of paraneoplastic encephalitis in association with recurrent neuroblastoma (NB) is rare. Here, we report a case of recurrent NB presented as paraneoplastic encephalitis, treated successfully with high dose steroids leading to complete neurological recovery. This case highlights the importance of early recognition, diagnosis, and treatment of paraneoplastic encephalitis in a pediatric patient with the new development of neurological symptoms in the background of NB. Paraneoplastic encephalitis also served as an early sign of disease relapse.

Rare case of phenytoin induced acute generalized exanthematous pustulosis with cerebellar syndrome.

Acute generalized exanthematous pustulosis (AGEP) is a rare drug induced cutaneous hypersensitivity reaction characterized by sudden onset of fever with sterile pustules overlying an erythematous skin occurring all over the body. The offending drugs are usually B-lactams and macrolides. Among anticonvulsants carbamazepine and Phenobarbital are commonly associated with AGEP. Only one case of phenytoin induced AGEP has been reported in literature. We present a rare case of AGEP with cerebellar syndrome occurring after receiving loading dose of phenytoin.