Long period of relative quiescence in distal-type epithelioid sarcoma of the forearm with recurrence after surgery: A case report.

BACKGROUND: Epithelioid sarcoma (ES) is a rare malignant mesenchymal tumor that only accounts for 0.6% to 1.0% of all cases of sarcomas. ES with a relative quiescent state of more than 10 years is extremely rare.Here, we present a rare case of ES in the forearm of a 17-year-old girl. The patient had a congenital mass in her forearm that measured approximately 1cm; it grew rapidly starting 5 years ago. The mass was not treated until last year when she underwent the first surgery. The mass was located in the middle and lower part of the left forearm and involved the dorsal muscle group, intermuscular space, and subcutaneous tissues without clear boundaries.The patient underwent surgery, and the tumor recurred twice within 1 year postoperatively. METHODS: The tumor samples were examined via hematoxylin-eosin (HE) and immunohistochemistry staining. RESULTS: Histopathologically, the tumor comprised large polygonal epithelioid cells with abundant eosinophilic cytoplasm arranged in cell nests. Central necrosis and focal myxoid change could be seen in the tumor tissues. Immunostaining showed that the tumor cells were positive for CD34, CK, EMA, and vimentin but negative for CD31, S-100, and INI-1. CONCLUSION: Based on these findings, the tumor was diagnosed as ES of distal form. Distal-type ES could have a long period of relative quiescence, after which it could grow rapidly and relapse multiple times over a short duration.

Congenital tri-cavernous hemangiomas of the right buccal region, right accessory parotid gland, and masseter muscle region.

We report a rare case of congenital tri-cavernous hemangiomas of the right buccal region, right accessory parotid gland, and masseter muscle region in an adult. The patient, a 25-year-old woman, complained of 3 masses in her right midcheek. Ultrasonographic and computed tomographic findings showed an irregular-shaped mass (multiple calcifications) with a well-defined margin in the masseter muscle region, an ellipse-shaped mass (multiple calcifications) with a well-defined margin in the right buccal region, and a comma-shaped mass (no calcifications) with a well-defined margin separate from the parotid gland in the right accessory parotid gland region. These iconographic findings suggested that the masses were all hemangiomas separately originating from the parotid gland, accessory parotid gland, and masseter muscle. The masses were completely removed through a standard parotid incision without postoperative facial palsy, skin deformity, and difficulty in secreting saliva. Findings from histologic examination of the tumor revealed multiple, thin-walled, and dilated blood vessels, confirming the diagnosis of cavernous hemangiomas. Ultrasonographic and computed tomographic findings were extremely useful in diagnosing the mass/masses as hemangioma before surgery, clarifying relationships between the mass and adjacent structures, and determining the surgical approach to the mass/masses.

Congenital smooth muscle hamartoma of the palpebral conjunctiva.

Smooth muscle hamartoma is defined as a disorganized focus or an overgrowth of mature smooth muscle, generally with low capacity of autonomous growth and benign behavior. The implicated tissues are mature and proliferate in a disorganized fashion. A healthy 5-day-old Mexican boy was referred to the authors' hospital in México city for evaluation of a "cystic" lesion of the right eye that had been noted since birth. The pregnancy and delivery were unremarkable. On physical examination, there was a reddish-pink soft lesion with a tender "cystic" appearance, which was probably emerging from the upper eyelid conjunctiva, which measured 2.7 cm in its widest diameter and transilluminated. Ultrasound imaging revealed an anterior "cystic" lesion with normally formed phakic eye. An excisional biopsy was performed, and the lesion was dissected from the upper tarsal subconjunctival space. Subsequent histologic and immunohistochemical findings were consistent with the diagnosis of congenital smooth muscle hamartoma (CSMH) of the tarsal conjunctiva. The authors' research revealed that only one case of CSMH localized in the conjunctiva (Roper GJ, Smith MS, Lueder GT. Congenital smooth muscle hamartoma of the conjunctival fornix. Am J Ophthalmol. 1999;128:643-4) has been reported to date in the literature. To the best of the authors' knowledge, this current case would be the second case reported of CSMH in this anatomic location. Therefore, the authors' recommendation is to include CSMH in the differential diagnosis of a cystic mass that presents in the fornix and palpebral conjunctiva.

Extremity salvage with a free musculocutaneous latissimus dorsi flap and free tendon transfer after resection of a large congenital fibro sarcoma in a 15-week-old infant. A case report.

A case of complex microsurgical reconstruction of the dorsum of the foot, including tendon transfer following tumor resection, in a 15-week-old male infant is presented. After birth, a 5.5 x 4 cm large tumor was observed on the dorsum of the right foot. Biopsy showed a congenital malignant fibro sarcoma. After initial chemotherapy a radical excision of the tumor at the age of 14 weeks was followed. To cover the defect a musculocutaneous latissimus dorsi flap was taken, the cutaneous part being large enough to cover the defect. Extensor tendons were reconstructed with free tendon transplants. Amputation is usually indicated in these cases. To the best of our knowledge, microsurgical reconstruction in infants at this age with congenital malignant tumors has not yet been reported. The case shows that Plastic surgery can play an important role in pediatric oncology and should routinely be integrated into the multi-modal treatment concepts.

Diaphragmatic lipoma in a 4-year-old girl: a case report.

A lipoma of the diaphragm is extremely rare. Although most congenital diaphragmatic lipomas are encountered in middle or old age because of their typical asymptomatic nature, none have been reported in patients younger than 14 years. We report the case of a large diaphragmatic lipoma in a 4-year-old patient.

Congenital neuroblastoma arising in the deltoid muscle.

Neuroblastoma almost always arises in association with sympathetic neural crest tissues that flank the spinal axis, including the paravertebral sympathetic ganglia of the neck, mediastinum, retroperitoneum, and pelvis, or in the adrenal glands. Neuroblastoma in the newborn period, which can present as localized or metastatic disease, often resolves spontaneously and requires little or no therapy. The authors describe a 5-week-old infant with an isolated primary neuroblastoma arising in the deltoid muscle. Histologic and biologic characteristics were consistent with a favorable-prognosis stage 1 neuroblastoma. Following a complete local excision, the child remains in complete remission 3 years from diagnosis.

Successful treatment of life-threatening generalized infantile myofibromatosis using low-dose chemotherapy.

Infantile myofibromatosis is the most common fibrous tumor of infancy. The generalized form of the disease is associated with a high rate of early mortality, especially if visceral structures are involved. Various therapeutic strategies have been used in these patients, including high-dose chemotherapy, with the risk of therapy-related toxicity. The authors present two cases of generalized infantile myofibromatosis, with life-threatening visceral and nonvisceral involvement, in which the patients were cured with a combination of low-dose chemotherapy and intensive care. The authors propose a prospective international trial using a safe low-dose chemotherapy protocol to test the efficacy of this treatment strategy.

Congenital and late-onset primary haemangiomas of the temporalis muscle: case report.

We report on two patients with haemangiomas of the temporalis muscle, one congenital and one of late onset, which were diagnosed on computed tomography. Both were resected successfully and both patients are well with no signs of recurrence 3(1/2) years and 18 months later, respectively.

Congenital infiltrating lipoma of the upper limb in a patient with von Willebrand disease.

Infiltrating lipoma is a rare variety of lipoma, characterized by an infiltration of the adipose tissue of the muscles. Infiltrating lipomas are usually classified in two groups: intermuscular infiltrating lipoma and intramuscular infiltrating lipoma. Most are acquired, and they usually appear in middle-aged individuals. Exceptionally, they are congenital. In such cases they are not related to other diseases. We report an 8-year-old boy with a congenital infiltrating lipoma of the upper limb and von Willebrand disease. Both diseases are linked to an alteration in chromosome 12, but this clinical association seems to be random rather than causal.

A case of congenital multiple myofibromatosis developing in an infant.

BACKGROUND: Infantile myofibromatosis is marked by the development of firm, discrete, flesh-colored to purple nodules in skin, muscle, bone, and/or subcutaneous tissues. In cases without visceral involvement, the prognosis is excellent with expected spontaneous regression of nodules in 1 to 2 years. Visceral lesions are associated with significant morbidity and mortality generally within the first few months of life secondary to obstruction of a vital organ, failure to thrive, or infection. OBSERVATION: We present a case of congenital myofibromatosis initially presenting as a single, asymptomatic nodule on the midback of an otherwise healthy 1-month-old white boy. Over the subsequent 6 months, the child developed a left-sided head tilt with the formation of additional myofibromas of the skin and musculature, but without visceral involvement. Physical examinations have continued to show age-appropriate growth and development. CONCLUSION: Clinicians should be aware of this rare but potentially life-threatening entity and consider infantile myofibromatosis in the differential diagnosis of pediatric dermal and subcutaneous nodules, particularly when associated with a new onset of head tilt. Close clinical follow-up is recommended in all cases of infantile myofibromatosis.

[Congenital diaphragmatic cyst in a child]. / Vrozená bránicní cysta u dítete.

The case of 8-year-old boy with congenital diaphragmatic cyst is presented. The aspects of the origin of this anomalies, the surgical finding and the method of the reconstruction of diaphragma are described.

Angiomyolipoma located in the lumbar region of a newborn.

An unusual example of angiomyolipoma, which presented as a bulging mass on the right lumbar region of a newborn baby, is presented. The case described is the first report of a newborn with an unusually located angiomyolipoma. The most common predilection sites are the renal parenchyma and the retroperitoneum. However, most of the patients described are adults, and to the best of our knowledge no newborn patient has previously been reported in the literature.