53-year-old female patient with a history of latent tuberculosis infection in prophylactic treatment with isoniazid, previous surgery of tonsillectomy, appendectomy and cholecystectomy. Family history negative for neoplastic diseases. Hospitalised at another institute in April 2016 with clinical manifestation of intestinal obstruction with evidence for CT scan of a solid occluding mass of a distal ileus of 5 cm maximum diameter localised in the right iliac fossa. Multiple nodular neoformations at the peritoneal level, the largest of 2 cm maximum diameter with associated free fluid in the abdomen and multiple lymphadenopathies of the ileal mesentery and some enlarged lymph nodes of the right anterior heart-phrenic angle. The latest was the unique finding of extra-abdominal disease spread. So the patient underwent surgery for ileal resection and ileostomy, with a peritoneal nodule biopsy.
The definitive histological examination of the ileal mass (free surgical resection margins) characterization showed positive reactions for histiocytic markers (CD163 and CD68); negative reactions for lymphoid markers (CD45 / LCA, CD20 / L26, CD79a, CD3, CD2, CD5, CD7, CD8, CD56, CD30 / BERH2 and ALK1) and myeloperoxidase. The reaction for cNPM is negative. Negative reactions for cytokeratins (MoAB MNF116, CK8,18, AE1 + AE3), desmin, caldesmon, ML actin, CD34, CD117, DOG1, S100 protein, melanA, CD21, CD1a. Cytoproliferative activity (MIB1-LI) in 15-20% of cells. Biopsy histological examination on the major of the peritoneal nodularity confirms the diagnosis of HS peritoneal localisation. The patient evaluation was performed with Esophagogastroduodenoscopy and Colonoscopy that excluded the involvement of other gastrointestinal tracts, bone marrow biopsy to confirm the absence of concomitant lymphoproliferative diseases. PET-CT showed multiple uptakes at the ileal mesentery, right and left colon, lymph node uptake also at the thoracic level at the phrenic heart angle, a finding already known in previous exams. The patient then performed six cycles of CHOEP chemotherapy. After adjuvant therapy, the PET-CT described a partial response to treatment with regression of uptake areas at thoracic level and persistence of abdominal uptake of already knows localisations (Fig. 1). After a multidisciplinary evaluation, was indicated the CRS: at the exploration of the abdominal cavity was established the disease recurrence from 15 cm to ileostomy, in the pelvis and along the left colonic mesentery and parietal peritoneum until homolateral hypochondrium, near the spleen. So the decision it was to perform an ileal en-bloc resection of the previous ileostomy, total colectomy, hysterectomy and bilateral oophorectomy, bilateral obturator lymphadenectomy, omentectomy, splenectomy, peritonectomy of all four abdominal quadrants removing multiple peritoneal nodules of the ileal mesentery. The spleen was removed to guarantee the radicality of surgery considering the presence of nodular lesions on the diaphragmatic peritoneal surface where the spleen leans. Peritoneal Cancer index was 13; at the end of the procedure, Cytoreduction score was CC-0.
Post-operative course was characterised by close monitoring in ICU up to the 3rd postoperative day and then transferred to our ordinary ward, the onset of infectious pneumonia resolved with antibiotic therapy. Discharge of the patient at home on the 20th postoperative day. Histological examination confirmed the diagnosis of HS with ileal relapse, multiple peritoneal, small bowel mesentery, colic and omental localisation, negative for HS localisation the other tissues removed. The postoperative CT scan showed a complete resection of abdominal HS localisation such as CT-scan at three and six mounths-follow-up. During the same period the patient underwent a second-line chemotherapy treatment with four cycles according to DHAP scheme (Dexamethasone, Cisplatin, Aracytin). The subsequent CT-scan performed at ten months from cytoreductive surgery (17 months from diagnosis) highlighted a disease relapse with multiple abdominal and thoracic localisations. Exitus at 21 months after diagnosis due to progressive decay of the general performance status.