A cerebriform connective tissue nevus (CCTN) is a key diagnostic feature of Proteus syndrome (PS), a rare genetic disorder characterized by the abnormal growth of bones, skin, adipose tissue, and other tissues. A plantar CCTN often causes difficulties with shoe fitting and walking. This paper presents a case of PS and the outcomes of debulking surgery for a plantar lesion. A 54-year-old male presented with a large plantar mass on his left foot, which caused problems with shoe fitting. The lesion developed during childhood and progressively worsened into adulthood. A physical examination revealed a typical CCTN with deep grooves and gyrations on the left plantar surface. The patient sought surgery due to difficulty in wearing shoes. Considering the postoperative complications associated with total excision and the potential risks associated with PS, including perioperative morbidities and anesthesia-related challenges, debulking surgery was chosen to reduce the lesion rather than perform a total excision. Staged excisions were performed three times under local anesthesia, with redundant tissue removed, and the wound was primarily closed after each procedure. At the one-year follow-up after the final procedure, the patient could comfortably wear ready-made shoes and exhibited an improved standing posture and reduced walking impairment. Therefore, debulking surgery for a large plantar CCTN can be an alternative to total excision.

Glomus tumors (GTs) are rare benign vascular neoplasms that predominantly occur in the subungual region of the digits. However, these neoplasms have also been reported in other anatomical locations. Extradigital GTs often present in atypical locations with unconventional symptoms, posing potential diagnostic challenges for clinicians. Herein, we present a recent case of an extradigital GT found in the forearm of a 76-yearold male patient that exhibited intraoperative features similar to those of a nerve sheath tumor or intravascular tumor, further underscoring these diagnostic challenges. This report highlights the pivotal role of frozen section pathology in diagnosing and managing this atypical lesion, thereby facilitating optimal patient care.

Pancreatitis, panniculitis, and polyarthritis (PPP) syndrome is a rare but critical disease with a high mortality rate. The diagnostic dilemma of PPP syndrome is the fact that symptoms occur unexpectedly. A 48-year-old man presented with fever and painful swelling of the left foot that was initially mistaken for cellulitis and gouty arthritis. The diagnosis of PPP syndrome was made based on the abdominal CT findings and elevated pancreatic enzyme levels, lobular panniculitis with ghost cells on a skin biopsy, and polyarthritis on a bone scan. The pancreatitis and panniculitis disappeared spontaneously over time, but the polyarthritis followed its own course despite the use of anti-inflammatory agents. In addition to this case, 30 cases of PPP syndrome in the English literature were reviewed. Most of the patients had initial symptoms other than abdominal pain, leading to misdiagnosis. About one-third of them were finally diagnosed with a pancreatic tumor, of which pancreatic acinar cell carcinoma was the most dominant. They showed a mortality rate of 32.3%, associated mainly with the pancreatic malignancy. Therefore, PPP syndrome should be considered when cutaneous or osteoarticular manifestations occur in patients with pancreatitis. Active investigation and continued observations are needed for patients suspected of PPP syndrome.
Abdominal Pain ; Anti-Inflammatory Agents ; Arthritis ; Arthritis, Gouty ; Biopsy ; Carcinoma, Acinar Cell ; Cellulitis ; Diagnosis ; Diagnostic Errors ; Fever ; Foot ; Humans ; Middle Aged ; Mortality ; Pancreatic Neoplasms ; Pancreatitis ; Panniculitis ; Skin ; Tomography, X-Ray Computed

Pancreatitis, panniculitis, and polyarthritis (PPP) syndrome is a rare but critical disease with a high mortality rate. The diagnostic dilemma of PPP syndrome is the fact that symptoms occur unexpectedly. A 48-year-old man presented with fever and painful swelling of the left foot that was initially mistaken for cellulitis and gouty arthritis. The diagnosis of PPP syndrome was made based on the abdominal CT findings and elevated pancreatic enzyme levels, lobular panniculitis with ghost cells on a skin biopsy, and polyarthritis on a bone scan. The pancreatitis and panniculitis disappeared spontaneously over time, but the polyarthritis followed its own course despite the use of anti-inflammatory agents. In addition to this case, 30 cases of PPP syndrome in the English literature were reviewed. Most of the patients had initial symptoms other than abdominal pain, leading to misdiagnosis. About one-third of them were finally diagnosed with a pancreatic tumor, of which pancreatic acinar cell carcinoma was the most dominant. They showed a mortality rate of 32.3%, associated mainly with the pancreatic malignancy. Therefore, PPP syndrome should be considered when cutaneous or osteoarticular manifestations occur in patients with pancreatitis. Active investigation and continued observations are needed for patients suspected of PPP syndrome.
Abdominal Pain ; Anti-Inflammatory Agents ; Arthritis ; Arthritis, Gouty ; Biopsy ; Carcinoma, Acinar Cell ; Cellulitis ; Diagnosis ; Diagnostic Errors ; Fever ; Foot ; Humans ; Middle Aged ; Mortality ; Pancreatic Neoplasms ; Pancreatitis ; Panniculitis ; Skin ; Tomography, X-Ray Computed

Eosinophilic gastritis is characterized by eosinophilic infiltration of the gastrointestinal tract, which is associated with abdominal pain, diarrhea, nausea, and vomiting. The possible etiologies of secondary eosinophilic gastritis, including drug reactions, parasitic infestation, and malignancy, must be evaluated. Herein we report the case of a 65-year-old North Korean defector who presented with nausea and vomiting for 1 year. Secondary (reactive) eosinophilic gastritis caused by cerebral sparganosis was suspected after a workup that included brain magnetic resonance imaging, cerebrospinal fluid tapping, and gastric mucosal biopsy. The patient showed dramatic clinical improvement with high-dose praziquantel treatment. Even though secondary (reactive) eosinophilic gastritis caused by parasites is very rare, this case shows the importance of considering parasitic infection in eosinophilic gastritis.
Abdominal Pain ; Aged ; Biopsy ; Brain ; Cerebrospinal Fluid ; Diarrhea ; Eosinophils* ; Gastritis* ; Gastrointestinal Tract ; Humans ; Magnetic Resonance Imaging ; Nausea ; Parasites ; Praziquantel* ; Sparganosis* ; Vomiting

We report a case of acute pancreatitis secondary to pancreatic neuroendocrine tumor. A 46-year old man presented with upper abdominal pain. The serum amylase and lipase were elevated. Abdominal computed tomography (CT) and magnetic resonance cholangiopancreatography revealed a 1.7 cm sized mass at the pancreas body with a dilatation of the upstream pancreatic duct and mild infiltrations of peripancreatic fat. An endoscopic ultrasound-guided fine needle biopsy was performed for the pancreatic mass, but only necrotic tissue was observed on the pathologic examination. A chest and neck CT scan revealed anterior mediastinal, paratracheal, and cervical lymph node enlargement, which were indicative of metastasis. An ultrasound-guided core needle biopsy was performed for the enlarged neck lymph node, and pathologic examination revealed a metastatic poorly differentiated carcinoma. Immunohistochemical analysis showed positive staining for synaptophysin, chromogranin A, and CD 56, indicative of a neuroendocrine carcinoma.
Abdominal Pain ; Amylases ; Biopsy, Fine-Needle ; Biopsy, Large-Core Needle ; Carcinoma, Neuroendocrine ; Cholangiopancreatography, Magnetic Resonance ; Chromogranin A ; Dilatation ; Endoscopic Ultrasound-Guided Fine Needle Aspiration ; Lipase ; Lymph Nodes ; Neck ; Neoplasm Metastasis ; Neuroendocrine Tumors ; Pancreas ; Pancreatic Ducts ; Pancreatitis ; Synaptophysin ; Thorax ; Tomography, X-Ray Computed

Ménétrier’s disease (MD), which is characterized by hypertrophic gastric folds and foveolar cell hyperplasia, is the most common gastrointestinal (GI) cause of protein-losing enteropathy (PLE). The clinical course of MD in childhood differs from that in adults and has often been reported to be associated with cytomegalovirus (CMV) infection. We present a case of a previously healthy 22-month-old boy presenting with PLE, who was initially suspected to have an eosinophilic GI disorder. However, he was eventually confirmed, by detection of CMV DNA using polymerase chain reaction (PCR) with gastric tissue, to have MD associated with an active CMV infection. We suggest that endoscopic and pathological evaluation is necessary for the differential diagnosis of MD. In addition, CMV DNA detection using PCR analysis of biopsy tissue is recommended to confirm the etiologic agent of MD regardless of the patient’s age or immune status.
Adult ; Biopsy ; Child ; Cytomegalovirus Infections ; Cytomegalovirus ; Diagnosis, Differential ; DNA ; Eosinophils ; Gastritis, Hypertrophic ; Humans ; Hyperplasia ; Infant ; Male ; Polymerase Chain Reaction ; Protein-Losing Enteropathies

No abstract available.
Adult* ; Humans ; Intussusception* ; Meckel Diverticulum* ; Muscle, Smooth* ; Pancreas*