Eccrine porocarcinoma is a rare malignant neoplasm of the eccrine sweat gland that often occurs in the lower extremities, and usually affects elderly individuals. Most cases of eccrine porocarcinoma arise de novo. We encountered a case of a large porocarcinoma arising in a pre-existing ganglion cyst in the knee. The malignant tumor was excised widely, and the defect was reconstructed using a free anterolateral thigh flap.
Aged ; Eccrine Porocarcinoma ; Ganglion Cysts* ; Humans ; Knee ; Lower Extremity ; Sweat Glands ; Thigh

It is uncommon for a palatal fistula to be detected in individuals who have not undergone surgery, and only sporadic cases have been reported. It is even more difficult to find cases of acquired palatal fistula in patients with submucous or incomplete cleft palate. Herein, we present 2 rare cases of this phenomenon. Case 1 was a patient with submucous cleft palate who acquired a palatal fistula after suffering from oral candidiasis at the age of 5 months. Case 2 was a patient with incomplete cleft palate who spontaneously, without trauma or infection, presented with a palatal fistula at the age of 9 months.
Candidiasis, Oral ; Cleft Palate* ; Fistula* ; Humans ; Oral Fistula

Temporal hollowing is a common complication that occurs after coronal approach surgeries. However, temporal hollowing without previous nerve damage or trauma history is rare. Herein, we present a patient with cryptogenic temporal hollowing. A 22-year-old man without any history of craniofacial interventions or trauma presented with temporal hallowing. Magnetic resonance imaging revealed fatty degeneration of the left temporalis muscle. Electromyography and nerve conduction study showed no signs of neurologic abnormalities. The patient received autologous fat injection of 30 mL harvested from the left thigh using the modified Coleman technique. Temporal hollowing is commonly caused by atrophy of the superficial temporal fat pad. Its incidence is reported to be as high as 6% after coronal approach operation. Augmentation using porous hydroxyapatite or titanium mesh is a treatment option. Autologous fat graft can also be an option for mild to moderate temporal hollowing. In this case, a patient with no history of trauma, surgery, or myogenic disease developed temporal hollowing. Further study of the littleknown cryptogenic form of temporal hollowing is warranted.
Adipose Tissue ; Atrophy ; Durapatite ; Electromyography ; Humans ; Incidence ; Magnetic Resonance Imaging ; Neural Conduction ; Thigh ; Titanium ; Transplants ; Young Adult

We present our experience with calvarial bone framework insertion through an intraoral approach for a patient who was at risk for columellar necrosis due to a previous open rhinoplasty. A 58-year-old woman exhibited severe columellar contracture, so that the columellar tissue was too fragile to be touched. We could not incise the columella and insert a new nasal implant through the bilateral rim incision. Moreover, the patient had septal cartilage perforation and collapse. The patient needed columellar support as well as nasal dorsum reconstruction. The authors decided to graft an autogenous L-strut bone framework through an intraoral approach. Two pieces of 5-cm × 1-cm sized split calvarial bone were harvested and trimmed to fit the width and length of the nasal dorsum and columella. The right-angle-shaped bone framework was made with an absorbable plate and screws. Through a gingivobuccal incision, the bone framework graft was inserted and the graft was fixed with absorbable screws. The patient did not experience complications such as skin necrosis or inflammation. A bone framework grafted through an intraoral approach can be a good choice for patients who have experienced scar contracture in the columella, septal cartilage perforation, and collapse.
Cartilage ; Cicatrix* ; Contracture* ; Female ; Humans ; Inflammation ; Middle Aged ; Necrosis ; Oral Surgical Procedures ; Rhinoplasty ; Skin ; Transplants*

The incidence of adrenal incidentalomas has increased because imaging studies are now being more frequently performed, including abdominal sonography, CT and MRI. Although there is only a consensus on the treatment of adrenal incidentalomas from the National Institute of Health (NIH) conference 2003, it is generally accepted that surgical resection is required if there's any possibility of malignancy or functionality of the adrenal tumor. Abdominopelvic actinomycosis is a rare chronic progressive suppurative disease that is caused by gram-positive bacteria of the genus actinomyces, which is part of the normal flora of the oral cavity and gastrointestinal tract, with low virulence. Herein, we report on a case of adrenal actinomycosis that imitated a huge adrenal tumor in a 39-year-old women, and the adrenal actinomycosis was confirmed histologically only after adrenalectomy. To the best of our knowledge, this is the first Korean case report on actinomycosis that occurred in the adrenal gland.
Actinomyces ; Actinomycosis ; Adrenal Glands ; Adrenalectomy ; Adult ; Consensus ; Female ; Gastrointestinal Tract ; Gram-Positive Bacteria ; Humans ; Incidence ; Mouth

Neurofibromatosis type 1 (NF1) is one of the most common autosomal dominant inherited disorders affecting the nervous system. NF1 is associated with mutations in the NF1 gene, which is located on chromosome sub-band 17q11.2 and contains 57 exons spanning approximately 300 kb of genomic DNA. NF1 is caused by a loss of function mutation of the NF1 gene, a tumor suppressor gene, which encodes for neurofibromin, a GTPase-activating protein (GAP) involved in the negative regulation of Ras activity. The GAP-related domain, which is encoded for by exons 20-27a, is one of the most important functional domains in neurofibromin. The cysteine-serine-rich domain has been recognized as an important functional domain in NF1-related pheochromocytomas. As the result of many genetic analyses of NF1-related pheochromocytomas, pheochromocytoma has generally been recognized as a true component of NF1. We recently experienced two families with NF1 accompanied by pheochromocytoma. The proband of family 1 is a 31-year-old female diagnosed with NF1 and pheochromocytoma. Gene analysis of the proband and her sister showed that the mutation of the NF1 gene (c.7907+1G>A) led to the skipping of exon 53 during NF1 mRNA splicing. The proband of family 2 is a 48-year-old male who was diagnosed with the same condition. Gene analysis demonstrated the mutation of the NF1 gene (c.5206-8C>G) with missplicing of exon 37. These novel germline mutations did not fall into the GAP-related nor the cysteine-serine-rich domains, but into the C-terminal area of the NF1 gene. This suggests that the correlation between the genotype and phenotype of NF1-related pheochromocytoma is somewhat difficult to characterize. Further studies will be necessary to confirm the function of the C-terminal area of the NF1 gene and its contribution to the development of NF1 and pheochromocytoma.
Adult ; DNA ; Exons ; Female ; Genes, Neurofibromatosis 1 ; Genes, Tumor Suppressor ; Genotype ; Germ-Line Mutation ; GTPase-Activating Proteins ; Humans ; Male ; Middle Aged ; Nervous System ; Neurofibromatoses ; Neurofibromatosis 1 ; Neurofibromin 1 ; Phenotype ; Pheochromocytoma ; RNA, Messenger ; Siblings