E. Simon

Pilomatrix Carcinoma of the scalp. A case report and review of the literature

INTRODUCTION Pilomatrix Carcinoma (PC) is a rare and malignant dermo-hypodermic tumor. Only 11 cases were reported in patients younger than 18 years old and only 13 cases were reported on the scalp. CASE REPORT We report the case of a 15-year-old woman who underwent cyst excision on the vertex. Anatomopathology shed light trichilemmal cyst. Five months later, she presented a first local recurrence. The tumor was removed with wide margin. Anatomopathology shed light PC. No adjuvant therapy was performed. The patient presented a second recurrence 3 months later with a parietal bone and superior sagittal sinus invasion and a lung metastasis. She underwent a craniotomy and radiochemotherapy. A third local recurrence was detected 4 months later. Three more lines of chemotherapy were performed without success. DISCUSSION PC is a locally aggressive tumour, with a high rate of local recurrences and metastases. PC arises de novo or through malignant transformation of a pilomatrixoma. PC were observed frequently in the white male over 50 years old. The histological diagnosis is difficult to prove. Treatment consists of a wide surgical excision. Peritumoral margins are not codified. Because of most cases are on the face and neck, Mohs Micrographic Surgery seems to be a good modality to limit margins. Radiation therapy is an adjuvant treatment. Chemotherapy can be used in metastasis case. CONCLUSION PC is a rare malignant tumor with high rate of disease relapse. Histological diagnosis is difficult and treatment is not standardized. Surgical procedure with wide margins is recommended to avoid the large recurrence when the staging shows no metastasis.

Particular catastrophic antiphospholipid syndrome, on the sole surgical site after breast reduction

A 20-year-old woman treated with vitamin K antagonist for antiphospholipid syndrome (APS) (pulmonary embolisms at age 15) was admitted for breast reduction after bridging therapy. At 2 days post-surgery haematomas appeared on the surgical site and anticoagulant therapy was withheld. She developed a skin and breast necrosis leading to the diagnosis of catastrophic APS. Despite medical treatment (anticoagulant therapy, corticosteroids and intravenous immunoglobulins) and surgery, necrosis continued. After 2 weeks of negative-pressure wound therapy (V.A.C.(®) Therapy™) the patient improved, mammary tissues were alive, well vascularised and budding. Breast reconstruction was then initiated. Artificial dermis graft (MatriDerm(®) 2 mm) was applied, and 3 weeks later the apposition of split-thickness skin graft on it. Six months later, results of the surgery were good and the patient was satisfied.

Incidence of occult contralateral carcinomas of the breast following mastoplasty aimed at symmetrization.

INTRODUCTION Breast carcinomas are the most frequent form of cancer in French women. Following a total mastectomy, only an estimated 25% of patients wish to undergo breast reconstruction. After mammary volume reconstitution, the plastic surgeon often attempts to harmonize the two breasts by carrying out contralateral reduction mammaplasty (CRM). In the literature, the incidence of occult contralateral carcinomas incidentally discovered in surgical specimens ranges from 1.12 to 4.5%. The main objective of this study was to evaluate occurrence of carcinoma in the CRM specimens in the framework of a breast reconstruction operation. The secondary objective was to determine the consequences of the incidentally discovered carcinoma in the contralateral breast. MATERIAL AND METHODS This was a 6-year, bicentric, retrospective study involving women having undergone breast cancer surgery who later underwent contralateral reduction mammaplasty (CRM), that is to say reconstruction aimed at harmonization of the two breasts. RESULTS Three hundred and nineteen patients were included in the study. Mean age during the CRM was 55years (29-79). Mean weight of the surgical specimens was 323grams (12-2500). Incidence of occult carcinomas found in the specimens was 0.94% (3 patients). The mean age for these 3 cases was 58years (47-64). All 3 patients had superior pedicle mammaplasty. One of the patients benefited from monobloc resection with orientation of the surgical specimen. In the other 2 cases, there existed 3 surgical resection specimens; in one case, they were oriented; in the other, they were not. In all 3 cases, the histological findings were unifocal ductal carcinomas in situ (DCIS). Mean tumor size was 5.7mm (3-9). Only the patient having had monobloc resection with orientation of the specimen underwent salvage surgery, which consisted in partial mastectomy, otherwise known as secondary lumpectomy. Adjuvant radiotherapy was administered to all of the patients. After 17months of mean follow-up (12-22), no recurrence was found in any of the three cases. CONCLUSION Incidence of occult contralateral breast carcinomas after symmetrization CRM approximates 1%. Our observations are in agreement with the data in the literature. Incidence is greater than in mammaplasty carried out for esthetic or functional reasons; this is probably due to the higher age and the previous breast cancer history of the breast reconstruction population. Monobloc resection and orientation of the surgical specimens with surgeons knots facilitate precise pinpointing of the occult carcinoma. A secondary lumpectomy may take place when margins of excision are invaded or inadequate.

New TASER injuries: lacrimal canaliculus laceration and ethmoid bone fracture

The TASER is a non-lethal conducted electrical weapon intended to incapacitate a person. The growing use of the TASER has resulted in an increased risk of injuries, including those to the face. We report a case of lacrimal canaliculus laceration and ethmoid bone fracture caused by an extra penetration (XP) TASER X26 dart. A 35-year-old was subdued with a TASER head shot; the probe was discharged into the left medial canthus without causing any ocular lesions. A computed tomography scan revealed the probe to be embedded in the left nasolacrimal duct and showed a displaced ethmoid fracture. The barbed dart had sectioned the inferior lacrimal canaliculus without electrifying the lesion. This case expands the knowledge of injuries that may occur as a result of the use of this device and the management of peri-ocular TASER injuries.

Cystathionine β-synthase genetic variant rs2124459 is associated with a reduced risk of cleft palate in French and Belgian populations

Background Orofacial cleft (OFC) is the most prevalent craniofacial birth defect. Genes involved in one-carbon, folate and vitamin B12 metabolisms have been associated with OFC but no study performed a concomitant assessment on genes involved in these three pathways. Objective We looked for potential genetic variants associated with OFC using an exhaustive gene panel of one-carbon metabolism. Methods We performed a case–control discovery study on children with OFC (236 cases, 145 controls) and their related mothers (186 cases, 127 controls). We performed a replication study on the top significant genetic variant in an independent group from Belgium (248 cases, 225 controls). Results In the discovery study on ‘mothers’, the CBS locus reached array-wide significance (p=9.13×10−6; Bonferroni p=4.77×10−3; OR 0.47 (0.33 to 0.66)) among the 519 haplotypes tested for their association with OFC risk. Within the CBS haplotype block (rs2124459, rs6586282, rs4920037, rs234705, rs234709), the rs2124459 was the most significantly associated with a reduced risk of OFC (p=1.77×10−4; Bonferroni p=2.00×10−2; OR 0.53 (0.38 to 0.74), minor allele). The rs2124459 was associated with a reduced risk of cleft palate (CP) (p=6.78×10−5; Bonferroni p=7.80×10−3; OR 0.40 (0.25 to 0.63)). In the ‘children’ group, the rs2124459 was associated with a reduced risk of CP (p=0.02; OR 0.61 (0.40 to 0.93), minor allele). The association between rs2124459 and reduced risk of CP was replicated in an independent children population from Belgium (p=0.02; OR 0.64 (0.44 to 0.93), minor allele). Conclusions The CBS rs2124459 was associated with a reduced risk of CP in both French and Belgian populations. These results highlight the prominent involvement of the vitamin B6-dependent transsulfuration pathway of homocysteine in OFC risk and the interest for evaluating vitamin B6 status in further population studies.

Zosteriform configuration of multiple eccrine spiradenomas of the scalp

BACKGROUND Eccrine spiradenoma (ES) is a benign adnexal tumor predominantly located in the head and neck regions. Multiple neoplasms located on the scalp have been reported but never with a zosteriform configuration on the first trigeminal area. CASE REPORT We describe an original case report of a 75-year-old Caucasian man presenting multiple subcutaneous blue and purple nodules disseminated on the first left trigeminal dermatome. All the nodules appeared gradually on a one-year period. Biopsy revealed a nodular adnexal tumor in the dermis without malignant eccrine spiradenoma (MES) transformation. The surgical procedure was performed in a manner to protect the galea aponeurotica in the upper half on the first left trigeminal area. The frontalis muscle was raised with the surgical specimen in the lower half of the first trigeminal area. A split-thickness skin graft was applied on the surgical defect. Histological examination revealed multilobular well-defined tumors located in the dermis. CONCLUSION The presence of multiple subcutaneous nodules in a trigeminal pattern should suggest a multiple localized zosteriform ES. The diagnosis is focused on clinical findings and the treatment is based on a large surgical excision. The histological examination is essential for not to fail a MES transformation.

Sarcome d’Ewing mandibulaire chez l’enfant : reconstruction par membrane induite

INTRODUCTION Ewings sarcoma (ES) is a malignant bone neoplasm that develops during the first two decades of life, and affects male more than female patients (sex ratio 1.6/1). ES head and neck bone localization is extremely uncommon (2 to 4%). We report a rapid induced membrane reconstruction without primary bone autograft. OBSERVATION A 7-year-old boy presented with a 50mm mandibular ES centered on the horizontal branch of the right mandible. This patient was treated by a combination of neo-adjuvant chemotherapy, surgery, and adjuvant radio-chemotherapy, according to the Euro-Ewing 99 protocol. The right horizontal mandibular branch was resected, following induction chemotherapy. A macroplate and a cement spacer were used for the reconstruction, while expecting anatomopathological results. Seventeen days later, we removed the spacer because of scar disunion. The radiographic controls revealed a spontaneous osteogenesis along the macroplate despite the early cement spacer removal. This spontaneously bone growth allowed avoiding a free vascularized bone transfer for the reconstruction. The tissue regeneration potential of this young boy and the cement spacer induced membrane could explain this spontaneous osteogenesis phenomenon. DISCUSSION Induced membrane can be added to the therapeutic options for pediatric oncologic mandibular bone loss. It avoids using of a free vascularized bone transfer.

Cas cliniqueSarcome d’Ewing mandibulaire chez l’enfant : reconstruction par membrane induiteEwing's sarcoma of the mandible in children: Reconstruction by induced membrane

INTRODUCTION Ewings sarcoma (ES) is a malignant bone neoplasm that develops during the first two decades of life, and affects male more than female patients (sex ratio 1.6/1). ES head and neck bone localization is extremely uncommon (2 to 4%). We report a rapid induced membrane reconstruction without primary bone autograft. OBSERVATION A 7-year-old boy presented with a 50mm mandibular ES centered on the horizontal branch of the right mandible. This patient was treated by a combination of neo-adjuvant chemotherapy, surgery, and adjuvant radio-chemotherapy, according to the Euro-Ewing 99 protocol. The right horizontal mandibular branch was resected, following induction chemotherapy. A macroplate and a cement spacer were used for the reconstruction, while expecting anatomopathological results. Seventeen days later, we removed the spacer because of scar disunion. The radiographic controls revealed a spontaneous osteogenesis along the macroplate despite the early cement spacer removal. This spontaneously bone growth allowed avoiding a free vascularized bone transfer for the reconstruction. The tissue regeneration potential of this young boy and the cement spacer induced membrane could explain this spontaneous osteogenesis phenomenon. DISCUSSION Induced membrane can be added to the therapeutic options for pediatric oncologic mandibular bone loss. It avoids using of a free vascularized bone transfer.