Primary pleuropulmonary synovial sarcoma with brain metastases in a paediatric patient: an unusual presentation

  Primary lung neoplasms are rare in children. The most common primary lung malignancies in children are pleuropulmonary blastoma and carcinoid tumour. Synovial sarcoma (SS) accounts for approximately 1% of all childhood malignancies. In absolute terms, the SS of the lungs and pleura are extremely rare and pose a diagnostic difficulty. Soft tissue sarcomas usually have a high potential for metastases, however, metastasis to the brain is rare, even in widely disseminated disease, and it has been described only in 3 case reports previously. Primary pleuropulmonary SS with brain metastases is even rarer. Here we present a case of an 11-year-old boy who presented with respiratory complaints, viz. fever and cough for 20 days. Initial impression was lung abscess, however, on histopathological, immunohistochemical and molecular study, the disorder was diagnosed as synovial sarcoma. After a week from the first consult, the child developed neurological symptoms, viz., an episode of convulsion and gradually worsening power of the lower limb. Computed tomography scan and Magnetic Resonance Spectroscopy was suggestive of brain metastases. Given the rarity of primary lung neoplasms in children, clinical detection remains a challenge. Delayed diagnoses are common as respiratory symptoms may be attributed to inflammatory or infective processes. Primary pleuropulmonary synovial sarcoma is a rare tumour and it is not known to commonly metastasise to the brain. Though rare, primary pleuropulmonary SS should be considered an important differential among peadiatric primary lung neoplasms due to its potential for curability if detected early, and more aggressive metastatic pattern, e.g. brain metastases making early detection imperative.

Chemo-resistant gestational trophoblastic neoplasia: a review of cases at a tertiary cancer centre

Background: Gestational trophoblastic neoplasia (GTN) was earlier a dreaded malignancy with high mortality rates. GTN is now considered to be one of the most curable solid tumours in women with cure rates greater than 90% even in the presence of metastases. Despite the high chemo sensitivity, treatment failure or drug resistance has been described in both groups.Methods: In this study, available records of GTN cases over 6 years were reviewed with emphasis on those who were resistant to the first line of chemotherapy. Of these, 37(34.58%) were resistant to the first line of chemotherapy. These cases were studied with respect to age, parity, antecedent pregnancy, interval from antecedent pregnancy, pretreatment β hCG, risk score and presence of metastases. The data was analyzed in order to find any risk factors associated with chemo-resistance.Results: Total number of cases of GTN was 107. Out of these 107 cases, 63 (58.88%) were low risk and 44 (41.12%) were high risk according to FIGO scoring system. Complete response was achieved with first line chemotherapy in 70 (65.42%) patients. The remaining 37 (34.57%) were resistant to first line chemotherapy. In the low risk group, 30 (47.62%) cases, and in the high-risk group, 7(15.91%) were resistant to first line of chemotherapy.Conclusions: Despite the high chemo sensitivity of GTN, resistance to first line chemotherapy may be encountered in up to 40% of cases.  It is important to identify the patients who are at risk to develop resistance, early identification of resistance and change of chemotherapy so as to minimize the exposure of these patients to ineffective chemotherapy

Pierwotny maziówczak opłucnej i płuca z przerzutami do mózgu u dziecka: nietypowe objawy

Pierwotny nowotwór płuc rzadko występuje u dzieci. Najczęściej spotykane pierwotne złośliwe nowotwory płuc u dzieci to blastoma opłucnej i płuc oraz rakowiak. Maziówczak stanowi około 1% wszystkich nowotworów złośliwych występujących u dzieci. Maziówczak opłucnej i płuca jest zjawiskiem rzadkim i trudnym do rozpoznania. Mięsaki tkanek miękkich zazwyczaj posiadają dużą skłonność do przerzutów, jednak przerzuty do mózgu występują sporadycznie, nawet w zaawansowanych postaciach rozsianej choroby. Taki przebieg choroby został przedstawiony zaledwie w 3 opisach przypadków. Pierwotny maziówczak opłucnej i płuc z przerzutami do mózgu jest jeszcze rzadziej spotykany. W niniejszej pracy przedstawiono przypadek 11-letniego chłopca, u którego pojawiły się gorączka i trwający od 20 dni kaszel. Początkowo sądzono, że przyczyną jest ropień płuc, lecz po wykonaniu badań histopatologicznego, immunohistochemicznego i molekularnego rozpoznano maziówczaka. Po upływie tygodnia od pierwszej wizyty u dziecka pojawiły się objawy neurologiczne, tj. drgawki i stopniowo postępujące osłabienie siły kończyny dolnej. Wyniki tomografii komputerowej i rezonansu magnetycznego wskazywały na istnienie przerzutów do mózgu. Biorąc pod uwagę rzadkość występowania nowotworów płuc u dzieci, rozpoznanie kliniczne choroby pozostaje prawdziwym wyzwaniem. Często zdarza się, że rozpoznanie jest opóźnione, ponieważ objawy ze strony układu oddechowego mogą być przypisywane stanom zapalnym lub chorobom zakaźnym. Pierwotny maziówczak płuc i opłucnej występuje rzadko i sporadycznie wywołuje przerzuty do mózgu. Powinien być jednak brany pod uwagę przy rozpoznaniu różnicowym pierwotnych nowotworów płuc u dzieci, ponieważ przy wczesnym rozpoznaniu może być uleczalny, zaś w przypadku agresywnego przebiegu z przerzutami na przykład do mózgu, wczesne rozpoznanie jest szczególnie ważne.Pierwotny nowotwór płuc rzadko występuje u dzieci. Najczęściej spotykane pierwotne złośliwe nowotwory płuc u dzieci to blastoma opłucnej i płuc oraz rakowiak. Maziówczak stanowi około 1% wszystkich nowotworów złośliwych występujących u dzieci. Maziówczak opłucnej i płuca jest zjawiskiem rzadkim i trudnym do rozpoznania. Mięsaki tkanek miękkich zazwyczaj posiadają dużą skłonność do przerzutów, jednak przerzuty do mózgu występują sporadycznie, nawet w zaawansowanych postaciach rozsianej choroby. Taki przebieg choroby został przedstawiony zaledwie w 3 opisach przypadków. Pierwotny maziówczak opłucnej i płuc z przerzutami do mózgu jest jeszcze rzadziej spotykany. W niniejszej pracy przedstawiono przypadek 11-letniego chłopca, u którego pojawiły się gorączka i trwający od 20 dni kaszel. Początkowo sądzono, że przyczyną jest ropień płuc, lecz po wykonaniu badań histopatologicznego, immunohistochemicznego i molekularnego rozpoznano maziówczaka. Po upływie tygodnia od pierwszej wizyty u dziecka pojawiły się objawy neurologiczne, tj. drgawki i stopniowo postępujące osłabienie siły kończyny dolnej. Wyniki tomografii komputerowej i rezonansu magnetycznego wskazywały na istnienie przerzutów do mózgu. Biorąc pod uwagę rzadkość występowania nowotworów płuc u dzieci, rozpoznanie kliniczne choroby pozostaje prawdziwym wyzwaniem. Często zdarza się, że rozpoznanie jest opóźnione, ponieważ objawy ze strony układu oddechowego mogą być przypisywane stanom zapalnym lub chorobom zakaźnym. Pierwotny maziówczak płuc i opłucnej występuje rzadko i sporadycznie wywołuje przerzuty do mózgu. Powinien być jednak brany pod uwagę przy rozpoznaniu różnicowym pierwotnych nowotworów płuc u dzieci, ponieważ przy wczesnym rozpoznaniu może być uleczalny, zaś w przypadku agresywnego przebiegu z przerzutami na przykład do mózgu, wczesne rozpoznanie jest szczególnie ważne

Genetic variability & chemotoxicity of 5-fluorouracil & cisplatin in head & neck cancer patients: a preliminary study

Background & objectives: The efficacy and toxicity of a given chemotherapy regimen varies widely among patients due to the inherited variability of genes that are involved in drug metabolism. There are several crucial enzymes identified involving metabolism of 5-fluorouracil (5-FU) and cisplatin, which are polymorphic. We studied head and neck cancer patients (n=23) on 5-FU and cisplatin combination therapy attending a tertiary care cancer research institute in Gujarat, India, to understand the effect of a particular genotype on toxicity. Methods: The patients were genotyped for dihydropyrimidine (DPYD) (85T>C, IVS14+1G>A, 2846A>T, 2194G>A), thymidylate synthase (TYMS) [28bp tandem repeat in the promoter enhancer region (TSER)], methylenetetrahydrofolate reductase (MTHFR) (677C>T, 1298A>C), glutathione S-transferase P1(GSTP1) (Ile105Val), glutathione S-transferase T1 (GSTT1) (null allele) and glutathione S-transferase M1 (GSTM1) (null allele) by multiplex allele-specific PCR and long range PCR. Results: Of the 23 (19 males 4 females, age range 18-16 yr) patients, two had grade 3 and 4 toxicity while the remaining 21 had 0 to 2 grade toxicity after treatment with 5-FU and cisplatin combination therapy. An association between the genotype of GSTM1 (+/- and -/-) and the toxicity of cisplatin (P=0.043) was observed. Interpretation & conclusions: The findings of this preliminary study suggested an association between the variants of GSTM1 and toxicity observed due to cisplatin. Well planned studies on a large sample of head and neck cancer patients need to be conducted to understand the effects of these genetic variants on toxicity and efficacy of anticancer drugs

Formulation development and evaluation of mouth dissolving film of domperidone

The present investigation was undertaken with the objective of formulating mouth dissolving film(s) of the antiemetic drug Domperidone to enhance the convenience and compliance by the elderly and pediatric patients. Domperidone is a drug of choice in case of nausea and vomiting produced by chemotherapy, migraine headaches, food poisoning and viral infections. It causes dopamine (D2 and D3) receptor blockage both at the chemoreceptor trigger zone and at the gastric level. It shows high first pass metabolism which results in poor bioavailability (10-15%). In view of high first pass metabolism and short plasma half-life it is an ideal candidate for rapid release drug delivery system. The solid dispersions of Domperidone were prepared with the use β-cyclodextrin in various ratios (1:1, 1:2, 1:3) and solubility study was performed to determine the ratio in which solubility of Domperidone was highest (1:3). The selected solid dispersions were then utilized for the preparation of film by solvent casting method utilizing HPMC E15 as a film forming agent and PEG-400 as plasticizer. Five formulae were prepared and were evaluated for their in vitro dissolution characteristics, in vitro disintegration time, and their physico-mechanical properties. The promising film (F1) showed the greatest drug dissolution (more than 75% within 15 min), satisfactory in vitro disintegration time (45 sec) and physico-mechanical properties that are suitable for mouth dissolving films

To Study Adverse Drug Reactions in MDR Pulmonary Tuberculosis Patients

Aim: To Study Adverse Drug Reactions In MDR Pulmonary Tuberculosis Patients. Methods: The Prospective Observational Study was carried out on patients of MDR/XDR Pulmonary Tuberculosis, registered at DR-TB Centre attended in OPD or admitted in DOTS Plus Site, Medical College, SSG Hospital, Vadodara for any adverse drug reaction, from January-2017 to October-2017. Total 80 MDR/XDR TB patients, who were registered at DR-TB Centre Baroda, were enrolled in the study for duration of 10 months. Results: Out of 80 patients most common Adverse drug reactions are gastro-intestinal upset 44/80 (55%), Arthralgia 14/80 (17.25%), Ototoxicity 12/80 (15%) and Psychiatric disturbances 5/80 (6.25%). Neuropathy 4/80 (5%), skin reactions 4/80 (5%), hypothyroidism 3/80 (3.75%) , and hepatotoxicity 3/80 (3.75%) also had been noted. Local toxicity, Visual disturbances, hypokalemia & Renal toxicity noted rarely. Out of 59 male patients most common Adverse drug reactions are gastro-intestinal upset (32 cases), Arthralgia (10 cases), Ototoxicity (10 cases) and Psychiatric disturbances(5 cases). Out of 21 female patients most common Adverse drug reactions are gastro-intestinal upset (12 cases), Arthralgia (4 cases), Ototoxicity (2 cases), Neuropathy ( 2 cases) and Skin reaction (2 cases). We had stopped the offending drug permanently in 16.3% of the patients. In 22.7% of patients, drug stopped upto recovery from adverse drug events. But in 61% of patients does not required the discontinuation of the offending drug. In some cases dosage of the drug is divided. As per the Preventibility Criteria According To Schumock And Thornton Scale, 62/80 (77.5%) ADRs are Not Preventable, 17/80 (21.25%) ADRs are Probably Preventable, and 1/80 (1.25%) ADRs are Definitely Preventable. Most common presenting symptoms are nausea-vomiting 44/80 (55%), Abdominal pain 18/80 (23%), Joint Pain 14/80 (18%), Hearing loss 12/80 (15%), and Breathlessness 7/80 (8.8%). Conclusion: Gastro-intestinal side effects which were commonest can be largely prevented by proper timing and spacing of drugs with food and if necessary, giving antiemetic, antacids and PPIs or H2 receptor blockers. These side effects are a common cause of defaulting and persuasive, sincere counseling is vital to help the patients through this ADR

A Study of Use of “PORT” Catheter in Patients with Cancer: A Single-Center Experience

Background: Effective and reliable venous access is one of the cornerstones of modern medical therapy in oncology. Materials and methods: This is a prospective observational study, which collected data of patients who require “PORT” catheter insertion for any cancer, at a tertiary care oncology hospital in Ahmadabad, Gujarat, India, during a 2-year period. Aims and objectives: The main objective of this study was to study the various complications and outcomes related to “PORT” catheters. Results: “PORT” catheter was inserted in 100 patients and was most commonly used in solid malignancies (n = 86, 86%), followed by hematologic malignancies (n = 14, 14%). Among the solid malignancies, breast cancer (38, 38%) was the most common underlying disease, whereas among the hematologic malignancies, acute lymphoblastic leukemia (6, 6%) was the most common underlying disease for “PORT” catheter insertion. Chemotherapy was started on the first day of “PORT” catheter in 74% of patients in the “PORT” study group. The various complications developed in the “PORT” study group in the descending order are as follows: 4 patients (4%) developed early infection (⩽30 days after “PORT” placement), 4 (4%) late infection (⩾30 days after “PORT” placement), 4 (4%) bloodstream infection, 2 (2%) local skin infection at the “PORT” insertion site, 2 (2%) dislodgment of the “PORT” catheter, 2 (2%) fracture of the “PORT” catheter, and 1 recurrent pleural effusion. One patient (1%) developed thrombosis as the complication of “PORT” catheter insertion. Conclusions: The most disturbing aspect of treatment for a patient with cancer is multiple painful venipunctures made for administration of cytotoxic agents, antibiotics, blood products, and nutritional supplements. The focus of this prospective observational research is to study the various underlying diseases for which “PORT” catheter is needed in different solid and hematologic malignancies and the various complications and outcomes in pediatric and adult patients with cancer

A comparative study of bloodstream infections in acute myeloid leukemia according to different phases of treatment: Can we predict the organism?

Introduction: The treatment of acute myeloid leukemia (AML) consists of induction therapy with anthracyclines and cytarabine followed by two to four cycles of consolidation therapy with high-dose cytarabine after achieving remission. There have been very few studies comparing infections during induction and consolidation. We have analyzed blood cultures of patients with AML during episodes of fever occurring during induction and consolidation, for comparing the bloodstream infections in both the phases. Materials and Methods: Blood cultures of patients during febrile episodes were collected from central venous catheters and peripheral blood, both during induction and consolidation therapy of AML. Results: The study population included 52 AML patients. During induction, there were 52 episodes of fever and 25 (48%) blood cultures were positive, 15 of these blood cultures reported Gram-negative organisms, 9 reported Gram-positive organisms and 1 as yeast. During consolidation, 47 episodes of fever were recorded and blood cultures were positive in 12, of which 7 were Gram-negative, 5 were Gram-positive. Conclusion: The incidence of blood culture positive infections during therapy of AML at our center was higher. The predominant organism isolated was Gram-negative both during induction and consolidation. The incidence of blood culture positive infections had decreased by 50% during consolidation