Extragonadal Germ Cell Tumor with Hepatic Infiltration in a Child: Aberrant Somatostatin Receptors and the Diagnostic Conundrum.

Extragonadal germ cell tumors (GCTs) are challenging to diagnose. We present a case of suprarenal GCT, with hepatic infiltration where differential diagnosis included neuroblastoma and hepatoblastoma. The positive positron emission tomography scan further obfuscated the situation. The diagnosis was clinched by fine-needle aspiration cytology and cell block immunohistochemistry.

Benign Renal Tumors in Pediatric Age Group: Retrospective Analysis.

BACKGROUND: Benign renal tumors are extremely rare and were studied here. This series also includes a renal teratoma in a horseshoe kidney, probably only the second in the pediatric literature. MATERIALS AND METHODS: Retrospective review of children with benign renal tumors operated between 2006 and 2018 at one center. RESULTS: Twelve patients (M:F ratio 10:2), age range 3 weeks (31-week gestation) to 13 years presented with large palpable renal swelling (n = 12) and hematuria (n = 3). Computed tomography (CT) scan showed features typical of the tumor. Final histopathology (age group [mean]) showed: multilocular cystic nephroma (MLCN) - n = 5 (41.7%), (11-16 months [13.6]); congenital mesoblastic nephroma (CMN) - n = 4 (33.3%) (classic 1, cellular 3) (0.75-5 months [2.125]); mature cystic teratoma - n = 1 (8.3%): (48 months, in a horseshoe kidney), and angiomyolipoma (AML) - n = 2 (16.7%) (144 months [sporadic] and 156 months [tuberous sclerosis]) One patient with cystic teratoma with no calcification on CT scan received pre-operative chemotherapy as fine-needle aspiration cytology (FNAC) reported malignant small blue cell tumor. Nephroureterectomy with Gerota's fascia could be done easily in all without intraoperative complications. Delay in presentation in MLCN and CMN led to increased symptoms and CT scan changes. All patients did well in 1.5-12 years (median 3 years) follow-up including cellular mesoblastic nephroma. CONCLUSIONS: Benign renal tumors often occur in specific age groups but may overlap that of Wilms tumor. Proper interpretation of clinical presentation, CT scan, and FNAC findings help in avoiding preoperative chemotherapy. Upfront nephroureterectomy is curative. Histopathological findings decide further treatment. Children with AML and tuberous sclerosis need lifelong follow-up.

Assessment of Clinical and Histological Changes in the Skin Paddle of Pectoralis Major Myocutaneous Flap in Intraoral Reconstruction: A Prospective Cohort Study.

PURPOSE: This study was carried out to characterize the clinical and histological changes in the cutaneous portion of the transferred pedicled pectoralis major myocutaneous flaps used in intraoral reconstruction in patients with head and neck malignancy. METHODS: This was a prospective cohort study carried out from July 2016 to 2018. All patients underwent ablative surgery for oropharyngeal cancers and primary reconstruction with pedicled pedicled pectoralis major myocutaneous flaps. The intraoral flaps were examined for color, texture, presence of hair, chronic inflammatory changes, and ulceration. At 12 months, incisional biopsies were taken from the skin paddle of the intraoral flap and contralateral normal buccal mucosa, and flap histology was compared with that of the contralateral buccal mucosa. RESULTS: Twenty patients were included in the final analysis (M/F, 4:1; mean ± SD age, 51.38 ± 6.76 years). Fourteen flaps resembled oral mucosa, 3 had a mixed appearance of both skin and mucosa, and 3 had appearance of normal skin at 1 year follow-up. The epidermis and stratum corneum were retained in all the flap biopsies; however, severe attenuation was noted in 7 patients (had mucosal appearance) but was significantly different from oral mucosa(P = 0.0003). Cutaneous appendages were found in all the flap epithelia. Thirteen flaps showed grossly attenuation, of which 11 patients had a gross appearance resembling oral mucosa and 2 had a mixed appearance. The biopsies showed varied degree of chronic changes like desquamation in around 35% (7 patients), hyperkeratosis in 35% (7 patients), and chronic candidiasis in 30% (6 patients). CONCLUSIONS: Although the intraorally transferred flaps demonstrate a morphological appearance similar to oral mucosa, there is a histological preservation of skin elements and architecture.

Outcome of antenatally diagnosed cystic hygroma - Lessons learnt.

PURPOSE: This study is carried out to characterize the prognosis and outcome of antenatally diagnosed cystic hygroma in a series of registered pregnancies. METHODS: This is a prospective cohort study, carried out over a period of 4 years (Jan 2016-Sept 2019). All the pregnancies referred from the department of obstetrics with antenatally detected cystic hygroma or increased nuchal thickness on level II ultrasonography suggestive of lymphangiomas were registered in the Pediatric Surgery outpatient department. Amniocentesis, fetal ECHO and fetal MRI (if indicated)were done. Prognosis was explained to the family and mode of delivery was planned as per the obstetric indications. Postnatal evaluation included general physical examination along with ultra sound (USG)doppler of the lesion. The neonates were admitted in neonatal surgical ICU for the administration of intralesional bleomycin in a dose of 0.3 IU/kg under strict observation. All the babies were followed up at 3, 6 and 12 months. Results were segregated as excellent, good and still in follow up cohort as per the final outcome. The fetal neck masses detected antenatally and the age at first dose of bleomycin were compared with the number of sclerotherapy doses required to achieve good response. RESULTS: Only nine patients out of 626 registered pregnancies (1.4%) were diagnosed with cystic hygroma. One antenatal mother opted for termination of pregnancy at 19 weeks of gestation and another patient was lost to follow up after receiving two doses of bleomycin in postnatal period. Maternal hypothyroidism was noted in two pregnancies. Antenatal USG shows increased nuchal thickness in 2 cases (first trimester) and 6 cases in level II scan. Amniocentesis shows normal fetal karyotyping in all these pregnancies. Fetal ECHO suggests normal biventricular function in 100% cases while fetal MRI done in one case completely ruled out any aerodigestive compromise. Mode of delivery is found to be planned elective LSCS in 57% of cases while normal vaginal delivery attained in 43% of cases as per the obstetric guidelines. Six cases achieved good response with >50% reduction in size out of which 4 cases received the dose at the first 10 days of life. Complete disappearance of lesion at the end of 6 months of follow up were seen in two babies. CONCLUSIONS: Antenatal screening for early detection of fetal cystic hygroma with possible associated congenital aneuploidies helps in prognostication and planning the mode of delivery. A multimodality approach during intra and postpartum increases safety margin. Even huge neck masses without associated anomalies carry fair prognosis. Intralesional bleomycin is safe and prevents surgical morbidity.