Giant Extraluminal Leiomyoma of the Colon: Rare Cause of Symptomatic Pelvic Mass

Leiomyomas (LMs) may appear throughout the entire gastrointestinal tract but are rarely seen in the colon-rectum and only 5 of those measured greater than 15 cm in diameter. Pain and palpable abdominal mass are the most common symptoms. Surgical resection is the treatment of choice for most LMs. We here describe a case of a 46-year-old woman who presented with a 3-month history of abdominal pain associated with worsening constipation and abdominal distension. A pelvic solid, polylobulate, left-sided mass was noted on examination. Preoperative findings revealed a dishomogeneous sigmoid mass with calcified spots compressing small intestine and bladder. At laparotomy, a large polylobulate and well-circumscribed mass arising from the descending colon mesentery and displacing small intestine, uterus, and ovaries. A segmental colon resection was performed. An extraluminal 18- × 12- × 5-cm paucicellular sigmoid colon leiomyoma was histologically diagnosed. Our case is one of the few giant (>15 cm) sigmoid colon LMs reported in the literature. Although rare and benign in nature, LMs of the colon can cause life-threatening complications that could require emergency treatment and they should be included in the differential diagnosis of large abdominopelvic masses. Follow-up after surgery is necessary for tumors with any atypia or mitotic activity.Key words: Leiomyoma, Digestive tract, Bowel obstruction, Pelvic massPrimary leiomyomas (LMs) present most commonly in the female genital tract and skin but they are rarely seen in the colon-rectum. Macroscopically, LMs may be intraluminal, intramural, extramural, or dumbbell.^1,2 The majority of LMs remain asymptomatic until they have reached a large size: most common symptoms are pain and palpable abdominal mass.^1,2 Symptomatic left colon LMs are uncommon and only 5 of those reported in the literature measured greater than 15 cm in diameter.¹ Histologically, most of them arise from the muscularis propria.^1,3 The most important differential diagnoses are malignant leiomyosarcoma and gastrointestinal stromal tumors (GIST).^1,3 Surgical resection is the treatment of choice for large LMs: complete excision should be always attempted and follow up is necessary for tumors with any atypia or mitotic activity.^1,2 Although rare and benign in nature, LMs of the colon can cause life-threatening complications that require emergency treatment.^1–3 We here describe a case of large extraluminal sigmoid colon LM causing pain and worsening bowel obstruction.     

Dose‐Intensified Granulocyte‐Monocyte Apheresis in Therapy Refractory Ulcerative Colitis

Granulocyte‐monocyte apheresis (GMA) is an emerging therapeutic option in active course of ulcerative colitis (UC). Appropriate GMA dose, including total number, frequency, and duration of the individual GMA session, is a matter of debate. It was the aim of the present study to evaluate the efficacy of a dose‐intensified GMA regimen in patients with moderately to severely active UC. A prospective open‐label, single‐center study was performed in 10 patients with active UC (Rachmilewitz Clinical Activity Index [CAI] ≥ 8 points; Rachmilewitz Endoscopic Index ≥ 7 points). Patients had failed to improve after treatment with steroids and/or immunomodulators. GMA was performed twice weekly for 2 h to a maximum of 10 sessions. In each GMA session, the adsorber was changed after 1 h of treatment time. Four patients achieved remission with a CAI ≤ 4 points. Three patients had a response with an improvement of CAI of ≥3 points. Three patients showed no benefit from GMA. The quality of life score determined by the inflammatory bowel disease questionnaire‐Deutschland increased by 26 points in median. First and second filters had similar efficiency in granulocyte and monocyte adsorption. No major adverse effects were observed. Dose‐intensified GMA as reported in this study provided an encouraging short‐term response rate of 70% in patients with moderately to severely active UC not responding to standard steroid or immunomodulator therapy. Although all patients relapsed not later than 16 weeks, GMA might be useful to reduce steroid and immunomodulator usage, or to delay surgery in this patient group.     

ER Stress During the Pubertal Growth Spurt Results in Impaired Long‐Bone Growth in Chondrocyte‐Specific ERp57 Knockout Mice

Long‐bone growth by endochondral ossification is cooperatively accomplished by chondrocyte proliferation, hypertrophic differentiation, and appropriate secretion of collagens, glycoproteins, and proteoglycans into the extracellular matrix (ECM). Before folding and entering the secretory pathway, ECM macromolecules in general are subject to extensive posttranslational modification, orchestrated by chaperone complexes in the endoplasmic reticulum (ER). ERp57 is a member of the protein disulfide isomerase (PDI) family and facilitates correct folding of newly synthesized glycoproteins by rearrangement of native disulfide bonds. Here, we show that ERp57‐dependent PDI activity is essential for postnatal skeletal growth, especially during the pubertal growth spurt characterized by intensive matrix deposition. Loss of ERp57 in growth plates of cartilage‐specific ERp57 knockout mice (ERp57 KO) results in ER stress, unfolded protein response (UPR), reduced proliferation, and accelerated apoptotic cell death of chondrocytes. Together this results in a delay of long‐bone growth with the following characteristics: (1) enlarged growth plates; (2) expanded hypertrophic zones; (3) retarded osteoclast recruitment; (4) delayed remodeling of the proteoglycan‐rich matrix; and (5) reduced numbers of bone trabeculae. All the growth plate and bone abnormalities, however, become attenuated after the pubertal growth spurt, when protein synthesis is decelerated and, hence, ERp57 function is less essential. © 2015 American Society for Bone and Mineral Research.