Ramani Manoj Kumar

Primary renal angiosarcoma

Primary angiosarcoma of the kidney is a rare tumor with only a few case reports in the literature. Management is not standardized and the prognosis is poor. However, clinicians need to be aware of this uncommon entity.

Peripheral primitive neuroectodermal tumor of the adrenal gland: A rare entity

Peripheral primitive neuroectodermal tumor (PNET) is an uncommon tumor and the overall incidence is 1% of all sarcomas. PNET of the adrenal gland is an even rarer entity. A 37-year-old female was evaluated for an episode of loin pain. Ultrasonography showed a large heterogenous left adrenal mass with internal echogenic components. Computed tomography did not show any fat density within to suggest a myelolipoma. Biopsy suggested a poorly differentiated neoplasm with a possibility of PNET of the adrenal gland.

Role of conventional and diffusion weighted MRI in predicting treatment response after low dose radiation and chemotherapy in locally advanced carcinoma cervix.

BACKGROUND AND PURPOSE To assess the diagnostic performance of conventional and diffusion weighted (DWI) magnetic resonance imaging (MRI) in predicting response in locally advanced cervical cancer. MATERIALS AND METHODS Total 24 patients with stage IIB-IIIB squamous cell carcinoma cervix were treated with initial two cycles of paclitaxel and carboplatin and concurrent low dose radiotherapy prior to standard chemoradiation. Response was assessed clinically and radiologically after 3 weeks of initial treatment. Volumetric and functional parameters derived from conventional and diffusion weighted MRI, due to treatment were measured. RESULTS Significant reduction of GTV was noted in MRI (54 cm(3) vs. 11 cm(3), p < 0.01) and DWI (44 cm(3) vs. 6 cm(3), p < 0.01, ΔADC = 0.49 × 10(-3)mm(2)/sec, p < 0.01) after treatment. Tumor volume reduction rate (TVRR) in DWI was significantly higher in pathological good responders (p = 0.03). In this group both mean post treatment apparent diffusion coefficient (ADC) value and ΔADC were significantly higher (p = 0.01 and p = 0.03). ADC was a good predictor for pathological response (area under receiver operating characteristic curve (ROC) 0.814). CONCLUSION TVRR (DWI) and ΔADC can be used as a predictor of early pathological response. Complete response based on DWI, could be a useful predictor of long term disease control.

Discriminating thyroid cancers from benign lesions based on differential expression of a limited set of miRNA using paraffin embedded tissues

BACKGROUND Micro-RNAs (miRNAs) are expressed in a tissue-specific manner and are known to demonstrate differential expression even among the various subtypes of a given tumor. This differential expression has been harnessed successfully in the development of diagnostic assays for various malignant tumors. These assays have been found to be relevant and of value as additional diagnostic tools even among thyroid tumors, particularly with regard to thyroid carcinomas of follicular morphology. MATERIALS AND METHODS A limited set of miRNA have been assessed as part of this study in an effort to use minimal number of miRNA markers (miR-187, miR-221, miR-222, and miR-224) to differentiate the benign from the malignant thyroid tumors using miRNA derived from paraffin embedded material. RESULTS While miR-221 and miR-222 were found to provide good accuracy as individual markers (86% and 84%), a combination of the two provided slightly better accuracy (91%). Both miR-221 and 222 were able to significantly differentiate malignant tumors from the benign samples (P< 0.001) individually and as a combination of markers. However, inclusion of miR-187 and miR-224 in the panel did not provide any additional benefit. CONCLUSION While a combination of miR-221 and 222 when used in a diagnostic panel could provide fairly good accuracy additional markers may need to be investigated to augment their diagnostic utility.

Second transurethral resection in T1G3 bladder tumors - Selectively avoidable?

Aim: To assess the need of a second transurethral resection (TUR) in select T1G3 bladder tumor patients. Materials and Methods: All the pT1G3 bladder tumors diagnosed during the period between January 2005 and December 2008 were included. Second TUR was routinely performed in all the pT1G3 bladder tumors within 4–6 weeks. Fifty out of the 68 patients with T1G3 underwent a second TUR and were retrospectively reviewed. The primary bladder lesions were grouped as solitary papillary, multiple papillary and sessile lesions. Statistical analysis was performed using STATA version 11 (STATA Corp., Texas, USA). Results: Forty percent (n = 20) of the lesions were solitary papillary, 48% (n = 24) were multiple papillary and 12% (n = 6) were sessile lesions. All our resections had muscularis propria sampled at the end of the resection and separately sent for histopathological examination (HPE), which showed them to be tumor free. Thirty-six percent of patients had residual disease at the second resection and 4% were upstaged. Ninety-five percent of the patients (n = 19) with solitary papillary lesions did not have any residual disease and 50% (n = 12) of the multiple papillary and 83.3% (n = 5) of the sessile group had residual disease at the second TUR. Conclusions: Patients with T1G3 tumors do not represent a homogenous group. Second TUR is recommended in patients with high-grade T1 urothelial bladder carcinoma as it identifies residual disease and invasive disease. Solitary papillary lesions may be the only group where the need for the second TUR is questionable.

Primary Renal Large Cell Neuroendocrine Carcinoma in a Young Man

Neuroendocrine tumours are usually located in the gastrointestinal or respiratory tract. A 23-year-old man was evaluated for loss of weight and a palpable left loin mass. CECT showed a large heterogeneously enhancing mass with calcification arising from the left kidney. He underwent an open radical nephrectomy. Histopathological examination revealed a large cell neuroendocrine carcinoma (LCNEC). LCNEC of the kidney is extremely rare and portends poor prognosis. It is usually a histopathological surprise and requires immunohistochemistry for confirmation.

Squamous cell carcinoma associated with large bladder calculus.

A 70-year-old male underwent an optical urethrotomy in 2010 for treatment of a dense 3-cm anastomotic urethral stricture. His history is significant for Gleason 9 T2b prostate cancer treated with an uncomplicated prostatectomy in 1998 and external beam radiotherapy in 2003 secondary to local recurrence. His urethral catheter was removed day 3 post-optical urethrotomy. He developed right groin pain radiating to the inner thigh and had difficulty weight bearing on the right leg over the subsequent days. His symptoms intermittently worsened and he presented 5 weeks post-optical urethrotomy to the emergency department with a fever, right groin pain and inability to walk. His C-reactive protein was elevated at 137 mg/L and a urine culture positive for pseudomonas aeruginosa. A contrast-enhanced computed tomography scan of his abdomen and pelvis revealed a hypoechoic lesion in his obturator externus muscle. An initial bone scan showed no area of increased uptake to suggest osteomyelitis. On day 3 of admission, a magnetic resonance imaging showed an extensive collection within the right obturator externus measuring 3.7 ¥ 2.1 cm as well as osteomyelitis of the pubis (Fig. 1). Ultrasound-guided drainage of the collection aspirated 3 mL of purulent material which grew Pseudomonas aeuroginosa. Optical urethrotomy is considered a safe and effective treatment for urethral strictures. The most common complications are fever, bleeding, urinary tract infection, epididymitis, urinary incontinence, urinary extravasation and recurrence of stricture. We postulate that a disruption of the anatomical planes secondary to radiotherapy resulted in the extravasation of urine, from a breach in the corpus spongiosum at time of urethrotomy, into the superficial perineal space which then tracked deep to Colles’ fascia into the medial compartment of the thigh. Pyomyositis is a rare but possible complication of optical urethrotomy, with patients previously treated with local radiotherapy at particular risk.

Angioleiomyoma of Uterus: A Clinicopathologic Study of 6 Cases:

Background and Objectives. Angioleiomyoma is a benign perivascular neoplasm commonly involving subcutaneous tissue of extremities, head, and trunk region. They rarely involve the female genital tract. This study analyses clinicopathological features of 6 cases of uterine angioleiomyoma. Methods. Routine sections of 6 cases were reviewed and immunohistochemical markers namely muscle-specific actin, h-caldesmon, desmin, CD10, WT-1, HMB-45, and melan-A were done. Results. Of the 6 cases, 4 cases had tumor involving the corpus and 2 cases had tumor in the cervix. Grossly, all tumors had a whorled and congested cut surface. Microscopic examination of all the cases revealed circumscribed neoplasms composed of interlacing fascicles of benign perivascular smooth muscle cells with evenly distributed slit-like blood vessels (solid variant) along with vessels exhibiting thick muscular walls with swirling pattern (venous variant). In only 2 cases many dilated vessels were seen (cavernous variant). Immunohistochemically, all cases were positive for muscle-specific actin, h-caldesmon, and desmin. All cases were negative for CD10 and WT-1 ruling out endometrial stromal tumor and were negative for HMB-45 and melan-A ruling out perivascular epithelioid cell tumor (both endometrial stromal tumor and perivascular epithelioid cell tumor have prominent vessels but have different histomorphology). In all cases, surgical excision was curative and there were no intraoperative or postoperative complications. Follow-up of all the cases has been unremarkable. Conclusion. As the World Health Organization has not included angioleiomyoma in the classification of mesenchymal tumors of uterine corpus and cervix, we recommend that it should be included in the classification.

Cutaneous metastasis of renal cell carcinoma masquerading as an infected sebaceous cyst

Renal cell carcinoma (RCC) is the third most common genitourinary malignancy with an estimated one third of cases with metastatic disease at the time of diagnosis. Though rare, cutaneous metastasis from RCC is more frequent than any other genitourinary malignancy. Metastasis of RCC to skin carries poor prognosis as coexistent visceral spread is the norm. A 38-year-old man presented one year after radical nephrectomy for clear cell carcinoma of the left kidney with a submental lesion that proved to be metastatic. Clinical appearance of cutaneous RCC metastasis is varied and can closely mimic other skin lesions. It is incumbent on general practitioners, dermatologists and urologists to exercise diligence in clinical diagnosis of skin lesions in the background of previous oncological diagnosis.

Squamous cell carcinoma of the scrotum: the revisit of a rare disease.

A 44-year-old farmer, with history of 10 pack-years of smoking, presented with a gradually enlarging painless ulcer on the right hemiscrotum for 1 year (Fig. 1). On physical examination, the penis and meatus were normal. A 7 × 6-cm irregular, non-tender, ulceroproliferative growth with necrotic base was seen over the right hemiscrotum. The margins were everted with minimal foul-smelling discharge. Base was free from the underlying structures. Small (10 × 5 mm), mobile, firm, inguinal lymph nodes were palpable bilaterally. Diagnosis of squamous cell carcinoma (SCC) was made on wedge biopsy from the margin of ulcer. He initially underwent wide local excision of the lesion with 2-cm healthy margin grossly. On histopathology, tumour was 3 mm from medial margin and 5 mm from superior margin. After 4 weeks, he underwent revision of the margins with bilateral ilio-inguinal lymph node dissection. Reconstruction was done using tensor fascia lata flap for groin and split thickness skin graft for the scrotal defect. One out of eight left inguinal nodes was positive while all right inguinal and bilateral pelvic lymph nodes were negative (Fig. 2). Post-operatively, he developed lymphocele, which settled with daily aspirations and prophylactic antibiotics. He is disease free after 1-year follow-up. SCC of the scrotum is the first occupational cancer described. In 1775, Perivall Pott first described it among the chimney sweepers in England. Scrotal carcinoma is a rare tumour with an overall annual incidence of nearly 1.5 per 1 000 000 persons in Western countries. Wright et al. collected 471 cases of scrotal cancer from 1973 to 2002 and found SCC (32%) to be the most common type followed by extramammary Paget’s disease (21%). Previously, environmental carcinogens such as chimney soot, tar, paraffin and some petroleum products were identified as the most commonly associated risk factors. Presently, most cases result from poor hygiene and chronic inflammation. It has been found that the tumour rarely involves the testis or the penis. Because of low incidence, there are no clear guidelines on the management of primary tumour and the inguinal nodes. Most of the literatures available are case reports only. Extrapolating the management guidelines on the SCC at other sites, we excised the primary lesion with 2-cm clear margin grossly. However, we had to revise the margin as clearance was less than 2 cm. It has been reported that in scrotal carcinoma, the surgical margin can still be positive despite wide excision with a 2-cm margin. Thus, it is important to ensure that a negative margin has been achieved during surgical resection to minimize the recurrence. Dai et al. reported 10 patients with scrotal carcinoma who were treated with wide surgical excision alone. It was seen that after an average follow-up of 47 months, eight patients were in good health without any relapse. Only one patient developed left inguinal lymph node metastasis at 21 months, which was successfully treated with bilateral inguinal lymphadenectomy. Various techniques like use of local thigh flaps, myocutaneous gracilis, adductor minimus myocutaneous flaps and heterologous fascia grafts are described to cover a healthy testis when primary closure is not feasible. In rare instances, some cases require orchidectomy to achieve closure. Because of the embryological development from the genital folds, the lymphatics of the scrotum do not cross the midline. Presti et al. advocated observation for clinically negative lymph nodes and prophylactic antibiotics for clinically palpable nodes. If