International medullary thyroid carcinoma grading system: an Indian tertiary care centre experience.

INTRODUCTION: Medullary carcinoma (MTC) is a rare neuroendocrine thyroid neoplasm. The international medullary thyroid carcinoma grading scheme (IMTCGS), which has prognostic significance, has been introduced recently. The present study graded MTC cases using the IMTCGS and evaluated it in our study cohort. METHODS: All MTC thyroidectomy cases over 6 years were evaluated. Low-grade (LG) and high-grade (HG) were compared. Survival analysis included overall survival (OS), loco-regional free survival and distant metastasis free survival (DMFS). RESULTS: Of 32 cases, 31.25% were HG and 68.75% LG. The mean age was 44.0 years and M:F ratio 1:1.146. HG patients were older and had tumour cells with high-grade nuclear features and prominent nucleoli and showed distant metastasis. Necrosis was found more in patients with high grade nuclear features. There was discordance between the high Ki67 (60%) and increased mitotic activity (20%). Univariate survival analysis revealed poor DMFRS and OS in the cohorts with high grade, Ki67 > 5% and coagulative necrosis. The multivariate cox regression analysis showed IMTCGS significantly associated with overall survival (HR 28.30, p = 0.009) and DMFS (HR 15.70, p = 0.02). DISCUSSION AND CONCLUSION: This is the first Indian study evaluating IMTCGS, a very simple and convenient grading system that can be readily used in any tertiary health care centre. IHC for Ki 67 should mandatorily be done irrespective of the low mitotic activity on the HPE and necrosis should be diligently searched in cases with high-grade nuclear morphology. HG MTC cohorts were associated with poor OS as well as DMFRS.

Posterior Sagittal Approach for Nonanorectal Malformation: Widening Spectrum.

Background: Posterior sagittal is a very well accepted approach in the treatment of anorectal malformations. This approach provides good access and exposure through the perineum to the deep pelvic structures. It reduces risk of injury to important structures as dissection remains in midline. Aims and Objectives: To access feasibility of posterior sagittal approach for non-anorectal malformation indications and to widen the spectrum. Material and Methods: We present a series of 10 cases of non-anorectal malformations operated by this approach for 4 years. Results: Six patients included in the study were of Disorders of Sexual Differentiation with pseudovagina, three of Y duplication of the urethra, and one of cervical atresia. All patients had good results. Conclusion: Posterior sagittal approach is feasible, safe with minimal bleeding, and no postoperative incontinence. It can safely be used for non-anorectal indications.

Retinoblastoma with Isolated Parotid Gland Metastasis: A Report of 2 Cases with Review of Literature.

Retinoblastoma, the most common primary intraocular malignant neoplasm in young children, show frequent metastasis to lymph nodes, central nervous system and bones. However, retinoblastoma metastasizing to parotid gland is very rare. We here present 2 cases of isolated parotid gland metastasis in two children with unilateral retinoblastoma. Case 1 was a one-year-old child presenting with left globe lesion which was diagnosed as Retinoblastoma. Post chemotherapy, enucleation was done which on histopathology examination showed retinoblastoma with scleral invasion and anterior chamber seeding. On 3 month follow up, patient presented with left parotid swelling. He underwent Fine needle aspiration cytology (FNAC) and Core needle biopsy which confirmed retinoblastoma metastasizing to parotid following which superficial parotidectomy was done as it was a solitary hotspot on PET-CT followed by localized radiotherapy and chemotherapy. While case 2, a 6-year-old child presented with left parotid swelling with a history of undergoing enucleation 3 months earlier in an outside hospital; Enucleation slides were reviewed in addition to patient undergoing FNAC and Biopsy from parotid swelling, all of which were consistent with Retinoblastoma metastasizing to parotid; This patient was lost to follow up; Although rare with only a few case reports in literature, both the pathologist and the clinician should be aware of retinoblastoma metastasizing to parotid, which when diagnosed and treated promptly has overall better prognosis in the cases reported so far.

Initial experience with 3D laparoscopic choledochal cyst (CDC) excision and hepatico-duodenostomy (HD) in 21 children.

INTRODUCTION: Minimal access surgery has gradually become the standard of care in the management of choledochal cysts (CDC). Laparoscopic management of CDC is a technically challenging procedure that requires advanced intracorporeal suturing skills, and hence, has a steep learning curve. Robotic surgery has the advantages of 3D vision, articulating hand instruments making suturing easy and thus is ideal. However, the non-availability, high costs and necessity for large-size ports are the major limiting factors for robotic procedures in the paediatric population. Use of 3D laparoscopy incorporates the advantage of 3D vision and at the same time allows the use of small-sized conventional laparoscopic instruments. With this background, we discuss our initial experience with the use of 3D laparoscopy using conventional hand instruments in CDC management. AIM: To study our initial experience in the management of CDC in paediatric patients with 3D laparoscopy in terms of feasibility and peri-operative details. MATERIALS AND METHOD: All patients under 12 years of age treated for choledochal cyst in a period of initial 2 years were retrospectively analysed. Demographic parameters, clinical presentation, intra-operative time, blood loss, post-operative events and follow-up were studied. RESULTS: The total number of patients were 21. The mean age was 5.3 years with female preponderance. Abdominal pain was the most common presenting symptom. All patients could be completed laparoscopically. No patient needed conversion to open procedure or re-exploration. The average blood loss was 26.67 ml. None of the patients required a blood transfusion. One patient developed a minor leak postoperatively and was managed conservatively. CONCLUSION: 3D laparoscopic management of CDC in the paediatric age group is safe and feasible. It offers the advantages of depth perception aiding intracorporeal suturing, with the use of small-sized instruments. It is thus a 'bridging the gap' asset between conventional laparoscopy and robotic surgery. LEVEL OF EVIDENCE: Treatment study level IV.

An Unusual Case of Type A Posterior Cloaca Associated with 46XX Disorder of Sexual Differentiation with Y Duplication of Urethra.

We report an infant reared as a girl who presented with ambiguous genitalia and urine coming per rectum. On examination, she had minimal clitoromegaly with labial fusion and Y urethral duplication. On investigations, genetic, metabolic, and endocrine causes for disorder of sexual differentiation were ruled out. This girl was operated via a posterior sagittal approach. This case was unique in that the urogenital sinus was deviated posteriorly and opened in the anterior rectal wall and the accessory urethra opened in clitoris, along with ambiguity of external genitalia, thus making it a rare variant of the posterior cloaca. The diagnosis requires a high index of suspicion in clinician and a meticulous examination of the external genitalia. Very little literature is available for this rare anomaly. Few reports have classified them as female pseudohermaphroditism with cloacal and urogenital sinus defects. Hence, we hereby discuss and review the literature for previously reported cases.

Giant Cutaneous Horn: Is It a Tip of An Iceberg? Two Case Reports and a Review of Literature.

Cutaneous horns are uncommon lesions which consist of proliferation of keratotic material resembling that of an animal horn. This lesion most commonly occurs in sun-exposed areas, particularly the face, ear, nose, forearms and dorsum of hands. The purpose of the study was to highlight the atypical presentation of squamous cell carcinoma as a giant cutaneous horn along with the review of literature. Case details were obtained from hospital records and clinical and histopathological findings were noted. Herein we report two cases of giant cutaneous horn associated with an underlying carcinoma presenting at an uncommon site. Both the patients were adults and had a chronic history of a skin lesion over the affected sites. Wide local excision was performed in both cases and histopathological examination confirmed the diagnosis of a cutaneous horn with a well differentiated squamous cell carcinoma at the base of the horn. Cutaneous horns are commonly associated with benign lesions and rarely with malignant conditions. It is imperative to biopsy the base of the horn to rule out any underlying malignancy. Proper surgical excision with adequate margins is the preferred treatment in case of an underlying malignancy.

Pseudovascular Adenoid Squamous Cell Carcinoma of Buccal Mucosa-a Rare Aggressive Variant.

Pseudo-vascular adenoid squamous cell carcinoma (PASCC) is an uncommon histological variant. It is characterized by an acantholysis of the tumor cells and the formation of anastomosing spaces and channels. It gives a false appearance of glandular differentiation mimicking angiosarcoma. PASCC has been reported in the head and neck, as well as in other organs like the breast, lungs, urinary bladder, vulva, and the uterine cervix. We report a case of PASCC of the oral cavity in a recently operated case of squamous cell carcinoma of the buccal mucosa.

Multidisciplinary Approach for Management of Pediatric Tumors: a Case Series.

Pediatric solid tumors have wide range of presentations. Multidisciplinary approach is often needed for their optimum management. There are no reports discussing such an approach to pediatric tumors involving pediatric surgeons, oncologists and cardiothoracic surgeons together for the management. We report 5 such cases being managed in our institution from 2010 to 2016. All cases needed chemotherapy followed by resection with a team of surgeons involving pediatric surgeon and cardiothoracic surgeon.

Posterior Sagittal Approach for Uterovaginal Anastomosis in a Case of Congenital Cervical Atresia with Anorectal Malformation.

Cervical atresia is a rare association with anorectal malformation (ARM) which can be missed till puberty in the presence of normal vaginal orifice. A 12-year-old girl operated for ARM in neonatal age presented with primary amenorrhea. She had a normal vaginal opening, short perineal body, and prolapsed anteposed anus and was diagnosed with cervical agenesis. As the posterior sagittal approach is standard to place the rectum in correct anatomical position, reconstruction of the anus along with adequate perineal body and uterovaginal anastomosis was performed through this approach. This report highlights the utility and versatility of this approach for the management of such complex cases.

Video-Assisted Thoracoscopic Surgery for Pediatric Empyema by Two-Port Technique: A Single-Center Experience with 167 Consecutive Cases.

BACKGROUND/PURPOSE: The aim of our study is to determine efficacy, safety, and feasibility of video-assisted thoracoscopic surgery (VATS) in childhood empyema with a technique of only two ports and open instruments at a tertiary care center in India. METHODS: This is a retrospective study of patients below 18 years, with empyema presenting to the Department of Pediatric Surgery of a Tertiary Care Referral Hospital in India, over a period of 9 years who underwent VATS decortication. Only two ports with open surgical instruments were used. The patients were assessed on the basis of mean duration of preoperative symptoms, duration of surgery, average blood loss, postoperative pain relief, complications, and need for redo surgery. RESULTS: A total of 97 patients underwent primary VATS decortications without inserting an intercostal drainage (ICD) tube and 70 patients as a secondary procedure after ICD tube was inserted. Mean duration of symptoms was 11 days. The average blood loss during surgery was estimated to be 170 cc. The mean duration of surgery was 90 min. The most common postoperative complication was air leak seen in 19.16% of patients. Minor leaks usually settled by 24 h. In eight patients, a negative suction had to be applied to the ICD tube for persistent air leak. The average length of postoperative stay was 4 days. Two patients required a repeat open decortication procedure due to nonresolution of symptoms and poor lung expansion after VATS. Patients had minimal pain and excellent cosmetic outcome after VATS. CONCLUSION: Two-port VATS decortication procedure is as feasible and effective as three-port procedure for decortication with better cosmetic result and pain relief.