Tamojit Chaudhuri

Primary clear cell adenocarcinoma of the uterine cervix in a young woman not associated with diethylstilbestrol: A case report and review of literature

We report an extremely rare case of primary clear cell adenocarcinoma (CCA) of the uterine cervix in a 21-year-old Asian female, with no maternal history of diethylstilbestrol ingestion during pregnancy. A review of the relevant literature regarding the possible etiology and treatment of primary CCA of cervix is done.

Is aspiration as detected on pretreatment video fluorography, a harbinger of poor quality of life and early mortality in cancers of the upper aerodigestive tract treated with radiotherapy?

Background: Patients with cancers of the upper aerodigestive tract (head and neck cancer (HNC)) tend to aspirate, either due to disease or treatment. The association of aspiration (documented on video fluorography (VFG)) with quality of life (QOL) and unexpected mortality was studied prospectively in patients treated with simultaneous integrated boost technique of intensity-modulated radiotherapy (SIB-IMRT). Materials and Methods: Moderately advanced (stage III/IV) HNC were treated by SIB-IMRT delivering 66 Gy/30 fr, 60 Gy/30 fr, and 54 Gy/30 fr to high, intermediate, and low risk volumes, respectively. They underwent serial VFG and QOL assessments (Quality of Life Questionnaire-Core 30 (QLQ-C30) and head and neck-35 (HN35) European Organisation for Research and Treatment of Cancer (EORTC) tools) at 0, 3, and 6 months. Pharyngeal musculature (PM) was additionally delineated on planning computed tomography (CT) scans as potential organs at risk (OARs). Results: Between November 2009 and May 2011, 20 HNC were treated as per protocol. All patients were fit (Karnofsky performance status (KPS) ≥ 80). Based on VFG findings, seven patients (4/9 oropharynx and 3/11 laryngopharynx) were grouped as aspirators (A) and remaining 13 as non-aspirators (NA). The QOL study showed that pretreatment coughing and swallowing difficulties were greater in group A versus NA and remained persistently higher. In group A, deaths attributable to aspiration were seen in 3/7 patients, while none occurred in the NA group (Fishers exact P = 0.03). The mean PM dose was 60 Gy in both the groups and mean V60 was similar at 69 and 67% in A and NA groups, respectively. Conclusions: VFG helps identify patients who aspirate and are at risk of premature death due to its complications, alerting caregivers to direct attention appropriately.

Association of cytochrome P450 2C9 polymorphism with locally advanced head and neck squamous cell carcinoma and response to concurrent cisplatin-based radical chemoradiation

Aims: The aim of the present study is to investigate the association between polymorphism of cytochrome P450 2C9 (CYP2C9) enzyme with head and neck squamous cell carcinoma (HNSCC) and response in patients receiving cisplatin-based radical chemoradiation (CT-RT). Materials and Methods: Four hundred and sixty patients suffering from locally advanced HNSCC and an equal number of healthy controls were genotyped for CYP2C9*2 and CYP2C9*013, leading to poor metabolizers (PMs) by polymerase chain reaction (PCR)-based restriction fragment length polymorphism (RFLP). Each case was assessed thoroughly for treatment response as per the World Health Organization (WHO) criteria. Results and Analysis: The frequency of heterozygous genotypes of both CYP2C9*2 (27.8%) and CYP2C9*3 (25%) were found to be significantly higher in the HNSCC cases as compared to the healthy controls. Tobacco intake in the form of chewing or smoking and alcohol intake resulted in several folds increase in the risk to HNSCC in the cases carrying variant genotypes of CYP2C9*2 or CYP2C9*013. Further, majority of the cases assessed for response (n = 436) carrying variant alleles of CYP2C9*2 (69.6%) or CYP2C9*3 (65.2%) were found to respond poorly to cisplatin-based radical CT-RT. Conclusion: The data suggests a significant association of the CYP2C9 polymorphism with HNSCC and treatment outcome underlining the importance of pretherapeutic genotyping in determining the treatment protocol.

Pilomyxoid astrocytoma of the thoracic spinal cord in an adult: A case report and review of literature

We present a case of pilomyxoid astrocytoma (PMA) in a 35-year-old Asian male with history of paraparesis for last 6 months. A contrast-enhanced magnetic resonance imaging of the spine revealed an intramedullary mass lesion occupying most of the thecal sac at the level of 10 th and 11 th dorsal vertebrae, with extensive contrast enhancement. Spinal PMA in an adult is an extremely rare entity, with only two reported cases in the literature, until date. This appears to be the first reported case of spinal PMA in an adult with isolated thoracic spinal cord involvement.

Male Breast Cancer Presenting with Bilateral Pleural Effusion

Dear Editor, Bilateral pleural effusion is a common clinical entity, but has never been reported as presentation of male breast cancer. The article reports about a 72-year-old Asian Indian male, who presented with breathlessness and anasarca since 1 year, progressively increasing since 1 month. The patient was breathless at rest and had a room air saturation of 86%. Personal history comprised of weekly intake of 5-6 pegs of whisky (approximately 100-120 g equivalent ethanol) since the past 30 years. The patient never smoked and had no history of illicit drug abuse, but had Type II diabetes mellitus and hypertension for past 5 years. General physical examination revealed icterus, bilateral pitting pedal edema, and a single left axillary lymph node, which was 2 cm in size, hard, mobile, and nontender on palpation. Respiratory system examination revealed enlarged breasts bilaterally on inspection and diminished air entry in the infra-scapular and infra-axillary regions on both sides. Palpation revealed a 3 × 2 cm nontender lump deep to the left nipple, not fixed to the chest wall. Abdominal examination revealed firm, irregular, nontender hepatomegaly (liver span 16 cm) and ascites. Hemogram and clinical chemistry were normal, except Hb (9 g/dl), s. bilirubin (3.1 g/dl), and albumin (2.2 g/dl) levels. Chest X-ray showed bilateral pleural effusion [Figure 1]. Ultrasonography (USG) whole abdomen revealed ascites and hepatomegaly with coarse echotexture. We did a therapeutic pleural fluid aspiration (800 ml from left and 1 lit from the right). Pleural effusion was exudative lymphocyte predominant with normal sugar and adenosine deaminase (ADA) levels on both sides. Left sided pleural fluid cytology was positive for adenocarcinoma cells. CECT thorax showed bilateral pleural effusion and fracture of rib. [Figure 2]. USG-guided fine needle aspiration cytology (FNAC) of left axillary lymph node was positive for metastatic ductal carcinoma. Core biopsy from the left breast lump revealed invasive ductal carcinoma [Figure 3]. A nodule of soft tissue density was seen in left breast [Figure 4]. Bone scan did not show any abnormal uptake. The patient was diagnosed as a case of metastatic invasive ductal carcinoma of left breast (cT2N1M1), and referred to Oncology department for further management. In view of poor general condition (ECOG 3), he was given palliative chemotherapy with fluorouracil, adriamycin (doxorubicin) and cyclophosphamide (FAC) regimen with 20% dose reduction (5-fluorouracil 500 mg/m2 i.v. on day 1, doxorubicin 50 mg/m2 i.v. on day 1, and cyclophosphamide 500 mg/m2 i.v. on day 1; repeated every 4 weekly). The patient died due to disease progression after receiving two cycles of chemotherapy. Figure 1 Scanogram showing bilateral pleural effusion Figure 2 Contrast-enhanced computed tomography thorax showing fracture of left rib Figure 3 High power view of breast nodule biopsy showing invasive ductal carcinoma Figure 4 Contrast-enhanced computed tomography thorax showing left breast nodule Breast cancer is the most common malignancy in females, but it is rare in males. Males comprise 0.7% of all breast cancer patients.[1] The etiology of male breast cancer comprises of hormonal, familial, genetic, and racial factors. Hormonal causes are increased estrogen-testosterone ratio (Klinefelter syndrome); increased estrogen levels (cirrhosis, exogenous administration in transsexuals, and prostate cancer therapy) and decreased androgen levels (testicular diseases like mumps and undescended testis or injury due to trauma or heat and electromagnetic radiation).[2] Familial predisposition occurs by presence of BRCA-2 gene mutations or a first degree female relative with breast cancer.[3] Genetic disorders like Cowden syndrome and hereditary nonpolyposis colonic cancer predispose males to breast cancer.[4] Racial predisposition for male breast cancer is seen in Jews and North American Black males.[5] Other predisposing factors are gynecomastia, alcohol intake of more than 60 g/day and obesity.[5] Bilateral gynecomastia may hide a malignant breast lump and unilateral gynecomastia may mimic one on clinical examination. Male breast cancer manifests as a hard, painless subareolar lump eccentric to the nipple. Pain occurs in 5% cases. Nipple retraction occurs in 9%, discharge in 6%, and ulceration in 6% cases. Pagets disease is the presenting feature in 1% cases.[6] Secondary features of malignancy are nipple retraction, skin ulceration or thickening, increased breast trabeculation, and axillary lymphadenopathy.[7] Invasive ductal carcinoma is the most common type of male breast carcinoma (90%). Other types include ductal carcinoma in situ (10%), invasive papillary (2%), medulllary (2%), and mucinous carcinomas (1%).[8] Ducts lie close to dermal lymphatics, pectoral fascia, and subareolar lymphatic channels in male breast. Due to lack of sufficient connective tissue, the ducts lie in close proximity to lymphatic system and metastasis occurs earlier in male breast. Estimates suggest 42% of breast cancer cases in men are diagnosed in advanced stages (stage III or IV).[9] Tumor size and lymph node involvement are the most important prognostic factors for male breast cancer. Other factors are histological grade and hormone receptor status. Expression of Her2-neu correlates with probability of increased relapse rates and higher stage and grade of the carcinoma.[2] Local disease is treated by modified radical mastectomy with axillary lymph node dissection or sentinel node biopsy.[9] Adjuvant chemotherapy is given for node positive disease. Tamoxifen is the therapy of choice for estrogen receptor positive cancer and metastatic disease.[10] An elderly male with co-morbidities (hypertension and diabetes mellitus), history of chronic alcohol intake, and presenting with anasarca and bilateral pleural effusion is usually suspected to have decompensated chronic liver disease or congestive heart failure, as they are most common differential diagnosis. Investigations revealed it to be a male breast cancer presenting with bilateral pleural effusion and pathological rib fracture. Malignancy should be considered among the differential diagnosis of bilateral pleural effusions in elderly.

Primary granulocytic sarcoma of lip - A rare extramedullary presentation of myeloid leukemia.

A sixty year old male presented with a swelling in the upper lip. On cytopathology, the patient was diagnosed as a case of granulocytic sarcoma. His bone marrow examination was unremarkable. Patient was then treated by radiotherapy alone 30 Gray in 15 fractions and achieved complete response. Eighteen months after follow up the patient is absolutely normal. To our knowledge this is the first ever reported case of granulocytic sarcoma of lip.

Trastuzumab-induced pulmonaryfibrosis: A case report and review of literature

Optimal treatment for human epidermal growth factor receptor 2 (HER2)/neu-positive, node-positive early breast cancer should include the monoclonal antibody trastuzumab. This relatively new targeted agent has shown very limited pulmonary toxicity. We report a case of Trastuzumab-induced pulmonary fibrosis in a 41-year-old female that occurred 4 months after starting adjuvant trastuzumab. To the best of our knowledge, this is the first ever report of trastuzumab-induced pulmonary fibrosis in the world of medical literature.

Primary central nervous system lymphoma in immunocompetent patients: A regional cancer center experience

Background: Primary central nervous system lymphoma (PCNSL) is a rare form of aggressive extranodal non-Hodgkins lymphoma which occurs in both immunocompromised and immunocompetent patients. It has an overall poor prognosis in spite of a multimodality treatment approach including chemotherapy and radiotherapy. This study attempts to further delineate the clinicopathological, immunohistochemical, and radiological profile of PCNSL at Kidwai Memorial Institute of Oncology, Karnataka, India. Materials and Methods: All the pathologically confirmed PCNSL cases between January 2010 and June 2016, at our center, were analyzed retrospectively. The influence of potential prognostic parameters on overall survival (OS) was investigated by log-rank test and Cox regression analysis. Results: Of the 26 PCNSL patients, 17 (65.3%) were males. Median age at diagnosis was 42.5 years. None of the patients had HIV or Epstein-Barr virus positivity and only four patients (15.4%) had B-symptoms. The most common location in the brain was cerebral hemispheres in 15 patients (57%) and 10 patients (38.5%) had multiple intracranial lesions. Histologically, all were diffuse large B-cell lymphomas, except one case of anaplastic large cell lymphoma. Immunohistochemically, 18 patients (69%) had MUM 1 positivity and 20 cases (77%) belonged to nongerminal center subtype. DeAngelis protocol was followed in 24 patients (92%), and among this cohort, Memorial Sloan Kettering Cancer Center Class 1 (n = 17) and Class 2 (n = 7) patients had a median OS of 25 months and 11 months, respectively. Conclusion: None of the potential prognostic factors had a statistically significant influence on OS in our patients. High-dose methotrexate combined with radiation is an effective therapeutic approach. However, further prospective studies with a large number of patients are needed to identify more effective primary chemotherapy regimens to further improve the treatment outcome.

Evolution of the treatment of primary central nervous system lymphoma in a Regional Cancer Center of South India: Impact of high-dose methotrexate on treatment outcome

Objective Primary central nervous system lymphoma (PCNSL) is a rare form of aggressive extranodal non-Hodgkin lymphoma. This study attempts to delineate the clinicopathological and radiological profile of PCNSL cases at our center. Materials and Methods All the pathologically confirmed PCNSL cases between January 2007 and July 2016 were analyzed retrospectively. The influence of potential prognostic parameters and therapeutic strategies on survival was investigated by log-rank test and Cox regression analysis. Results Of the 53 PCNSL patients, 34 (64%) patients were males. Median age at diagnosis was 44 years (range 22-65 years). The most common location in the brain was the cerebral hemispheres in 32 patients (60%), and 16 patients (30%) had multiple intracranial lesions. Histologically, all patients were diffuse large B-cell lymphomas, except one case of anaplastic large-cell lymphoma. The median survival of the patients received whole-brain radiation alone ( n = 6), standard CHOP chemotherapy + radiation ( n = 14), and DeAngelis protocol ( n = 31) was 8 months, 13 months, and 23 months, respectively. Among the 31 patients treated with DeAngelis protocol, Memorial Sloan Kettering Cancer Center Class 1 ( n = 23) and Class 2 ( n = 8) patients had a median overall survival (OS) of 25 months and 13 months, respectively. The incidence of treatment-related neurotoxicity was significantly higher with DeAngelis protocol, in comparison to CHOP + whole-brain radiation therapy (26% vs. 14%, P < 0.05). Conclusion None of the potential prognostic factors had a statistically significant influence on OS in our patients. High-dose methotrexate-based chemotherapy combined with radiation was the only factor, which had a significant impact on survival (log-rank P = 0.000) but at the cost of increased neurotoxicity.

Langerhans cell histiocytosis of atlantoaxial joint in a middle-aged man presenting with deafness as first symptom and soft-tissue mass at neck showing excellent response to radiotherapy alone: Report of an extremely rare and unusual clinical condition and review of literature

Langerhans cell histiocytosis (LCH) is a disorder of clonal proliferation of dendritic cell mainly occurring in children. Spine involvement is rare. This usually presents with pain and torticollis when neck is involved. Histopathology with immunohistochemistry is confirmatory. Local curative therapy with excision or curettage is used for localized disease. Radiotherapy is usually reserved for selected cases. Systemic chemotherapy is the treatment of choice for widespread systemic disease. In this article, we present an unusual presentation of atlantoaxial LCH with mastoid involvement resulting in hearing loss as the first symptom and quadruparesis in a middle aged male patient, which was also associated with soft-tissue mass at the nape of the neck and deafness. The patient was treated with radical radiotherapy, which provided excellent response to the disease. Involvement of atlantoaxial joint and temporal bone associated with soft-tissue mass neck and deafness in a middle-aged man is an extremely rare clinical situation.