Complex Surgical Oncology Case Series: Peutz-Jeghers Syndrome with Locally Advanced Hepatic Flexure Colon Cancer Treated at Shri Mahant Indiresh Hospital, Dehradun.

Understanding Peutz-Jeghers Syndrome (PJS)

Peutz-Jeghers Syndrome (PJS) is a rare genetic disorder characterised by the growth of benign (non-cancerous) polyps throughout the gastrointestinal (GI) tract. These polyps can cause abdominal pain, bleeding and obstruction. People with PJS also have an increased risk of developing various cancers, including cancers of the stomach, intestines, pancreas, lungs, breast, ovaries and cervix.

Case Overview

At the Department of Surgical Oncology, Shri Mahant Indiresh Hospital, we recently encountered a challenging and complex case involving a patient clinically diagnosed with Peutz-Jeghers Syndrome. The patient presented late in the disease course with adenocarcinoma of the hepatic flexure and severe locoregional spread, including infiltration into hepatic segments 5 and 6, the adjacent body wall, and colonic fistulisation extending to the lumbar body wall, adjacent diaphragmatic wall and 12th rib.

Due to the absence of obstruction, our team administered four cycles of neoadjuvant chemotherapy to facilitate prehabilitation and improve the patient’s performance status.

Surgical Intervention

Dr. Ajeet Tiwari, leading the surgical team, performed a staging laparoscopy to exclude miliary metastasis and peritoneal involvement, followed by a formal midline exploratory laparotomy and extended right hemicolectomy. This included an en-bloc resection of liver segments 5, 6 and part of segment 4. Entire Surgical Team’s expertise was crucial for precise anatomical preoperative planning, particularly to avoid injury to the duodenum despite dense adhesions. Major liver resection was done using a pseudo liver hanging technique in the constrained space between the tumour, segment 6, diaphragm, and Gerota’s fascia.

The surgical intervention further involved:

  • Resection of the body wall with the enterocutaneous fistula, the 12th rib
  • Ileocolic anastomosis using the Barcelona Technique
  • Closure of the body wall defect with an omental and thoraco-abdominal fasciocutaneous rotation flap
  • Cholecystectomy

Long term experience of the Surgical Team with thoracoepigastric, thoracoabdominal, and omental regional flaps enabled successful closure of the abdominal wall defect with minimal postoperative complications.

  • Operating Time: 6 hours
  • Blood Loss: 300 ml
  • ICU Stay: 4 days
  • Ward Stay: 3 days

Histopathology

The final histopathological report revealed ypT4bN0M0 (lymph node yield-30, all margins negative). The patient is currently undergoing adjuvant chemotherapy with close surveillance for Peutz-Jeghers Syndrome.

Challenges of This Case

  1. Multivisceral Resection Requirement: The extensive locoregional spread required resection of multiple organs and tissues.
  2. Complex Anatomical Considerations: Precise anatomical knowledge was essential to avoid injury to vital structures and ensure complete tumour removal.
  3. Borderline Performance Status: Prehabilitation and neoadjuvant chemotherapy were crucial in improving the patient’s condition before surgery.
  4. Financial Constraints: Managing the patient’s treatment within financial limitations posed an additional challenge.

Learning Points

  1. Precise Anatomical Knowledge: Essential for successful multivisceral resections and reconstructions.
  2. Proficiency with Flaps: Surgical oncologists should be skilled in using locoregional and pedicled flaps for reconstruction.
  3. Role of Prehabilitation: Critical for improving patient performance status before major surgery.
  4. Teamwork: Collaboration and support from all departments and administration are vital for successful outcomes. “Alone we go faster, but together we go farther.”

Dr Ajeet Tiwari’s leadership and the team’s collaborative efforts were instrumental in successfully managing this complex case. Dr Tiwari’s extensive experience with Complex Cancer Cases, along with the unwavering support from all departments and administration of Shri Mahant Indiresh Hospital, were paramount in achieving a positive outcome for the patient.

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