A 63-year-old male patient was referred to our Surgical Department from the Department of Dermatology with the diagnosis of a large retroperitoneal pelvic tumor for further treatment. The mass was morphologically consistent with a Castleman's tumor. Patient's symptoms had begun two years earlier with rapidly progressive diffuse cutaneous lesions all over the trunk and extremities as well as oral lesions with the form of erythematous plaques and superficial hyperceratosis. Erosive mucositis with lichenoid inflammation affecting the oral and penis glance mucosa as well as dystrophic nails associated with periungular erosions and onychorrexis had been observed. The patient had been initially treated with local corticosteroids and acitretin (Neotigason®) but skin lesions had remained stable or had shown a mild shortlasting remission only. The histological examination of the lesions was consistent with lichen ruber verucosus and pemphigus vulgaris.
One month before admission the patient was intravenously treated for an atypical pneumonia and alveolitis with Rocephin and Prednisolon (100 mg for 3 days). As skin lesions had shown no signs of remission, further diagnostics was initiated in order to determine a possible paraneoplastic nature of skin pathology.
A subsequently performed contrast-enhanced multislice CT demonstrated a highly vascularized retroperitoneal mass (10.3 × 9.2 cm) with significant contrast enhancement consistent with a sarcoma or a Castleman's tumor (Figure and ). The tumor filled the whole lower pelvis without any signs of adjacent organs' invasion though.
Figure 1 A). Preoperative CT scan of the abdomen showing a highly vascularized retroperitoneal tumor measuring 10 × 9.2 cm with intratumor calcifications. Right ureter dilatation (grade II), (coronal multiplanar reformation, MPR) B). The same tumor in (more ...)
On physical examination besides the above mentioned diffuse erosive muscositis with lichenoid inflammation, no lymphadenopathy or organomegaly was noted. Preoperative examination of the complete blood counts revealed the following results: hematocrite 41%, white blood cells 11.0/nl (80% neutrophils), PTL 562 k/m, PT 90% (INR 1.07).
Following thorough preoperative diagnostics the patient was submitted to laparotomy for resection of the tumor. After catheterisation of both ureters with "pig-tail" catheters a midline laparotomy was performed. Entrance into the peritoneal cavity revealed a highly vascularized solid mass occupying the small pelvis extending to the right iliac fossa.
After careful dissection of the right iliac vessels and right ureter off the mass, complete excision of the bulky tumor could be performed. Special attention was given to the meticulous ligation of the tumor vessels, mainly collaterals coming from the internal iliac artery, which was separately ligated.
After surgery the patient was treated for four days on the ICU. Postoperative course was uneventful, so that the patient could be referred to the Dermatology Department for further treatment of his skin lesions. A complete evaluation of the patient's immune status was performed. Main results are as following: lymphocytes 5%, CD3+ 61% (reference value 59.4–84.6), CD3, CD4/CD3, CD8 0.91 % (0.9–3.6), CD3/CD19 12% (6.4–22.6), CD3/CD4 30 % (28.5–60.5), CD3/CD8 33 % (11.1–38.3), CD3/CD16+56 23% (5.6–30.9). Anti-Desmoglein 1-Abs were 59.96 (>20 positive), anti-Desmoglein 3-Abs 48.25 (>20 positive), BP 180-Abs 6.25 U/ml (>9 positive). Skin Abs were negative, but circulating IgG antibodies reactive with rat urinary bladder epithelia surfaces were detected positive (diagnostic of paraneoplastic pemphigus). New histological examination of biopsies taken from skin lesions, extremities, and the oral mucosa were consistent with the diagnosis of lichen ruber and highly suggestive of a pemphigus. The latest diagnosis was confirmed with direct immunofluorescence, which showed a high intracellular deposition of anti-IgG (antibodies against desmosomes) in the whole epidermis. Histological examination of the mass revealed a Castleman tumor of hyaline vascular type locally in sano removed.
One month after surgery the patient was newly referred to us with a postoperative 15 × 11 cm pelvic abscess, which was percutaneously drained under CT control. Culture of the drained pus was positive for staphylococcus aureus (MRSA) and an intravenous treatment was initiated (linezolid and flucloxacillin). Percutaneous drainage was repeated two weeks later so that a rest fluid around the right psoas muscle could be successfully evacuated. Due to progression of the painful skin lesions an immunosuppressive treatment was initiated with cyclophosphamide and urbason (every two weeks) and cortison daily followed by a per os immunosuppression protocol with cyclophosphamide and endoxan.
A dramatic improvement of the skin lesions could be seen in a period of three weeks' time. Following a removal of the left ureter catheter and change of the right one, the patient could be released in a good condition with almost complete remission of the mucocutaneous lesions four weeks later (2 months after initial surgery).