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UNASSIGNED: The diagnosis of a pheochromocytoma or paraganglioma secreting during pregnancy is a rare and serious situation, involving maternal-fetal prognosis. The purpose of this case series is to discuss the management of these patients.
METHODS: This is a retrospective study of cases of pheochromocytoma (n=2) or paraganglioma (n=2) managed during pregnancy between 2013 and 2020 in one center (Lille, France).
RESULTS: We report four cases of patients with a diagnosis of pheochromocytoma or paraganglioma during pregnancy, at respectively 4, 28, 31 and 34 weeks of amenorrhea (AS). Their pregnancies were affected by a sudden onset of hypertension sometimes associated with headaches, sweating, and palpitations. All patients delivered by Caesarean section after calcium channel blocker impregnation, with a good outcome. Tumor removal took place at a distance from delivery for each patient.
CONCLUSIONS: The therapeutic strategy includes antihypertensive treatment with calcium channel blockers or alphablockers and surgical curative treatment linked to gestational age. Multidisciplinary management as well as early diagnosis can improve the maternal-fetal prognosis. The preferred way of delivery is Caesarean section, but vaginal delivery can also be considered. Removal should ideally take place at a distance from the birth. The analysis of these cases has led to the development of a protocol for monitoring and management of parturients with diagnosis of pheochromocytoma or paraganglioma during pregnancy.

Benign and malign tumors can affect the temporomandibular joint (TMJ) as any other articulation. Nevertheless, TMJ tumors are rare and mostly benign. Their clinical expression is varied including symptomatology similar to TMJ dysfunctional disorders, otologic or neurologic pathologies. In some cases, they remain totally asymptomatic. Hence, diagnosis is difficult since the symptomatology can be misleading with TMJ dysfunctional disorders or otologic disorders wrongly diagnosed. There is thus frequently a long delay between symptoms onset and diagnosis. The great variety of TMJ lesions explains the wide range of possible treatment modalities, mostly based on surgery. We provide here a review of the lesions originating from the TMJ. Tumoral or cystic mandibular lesion affecting the TMJ through local extension will not be discussed. Osteoma, osteoid osteoma, osteoblastoma, chondroma, osteochondroma, chondroblastoma, tenosynovial giant cell tumors, giant cell lesions, non-ossifying fibroma, hemangioma, lipoma or Langerhans cell histiocytosis are all possible diagnosis among the benign tumors found in the TMJ. Pseudotumors include synovial chondromatosis and aneurysmal bone cyst. Finally, malign tumors of the TMJ include mainly sarcomas (osteosarcoma, chondrosarcoma, synovial sarcoma, Ewing sarcoma, and fibrosarcoma), but also multiple myeloma and secondary metastases. We will review the clinical, radiological and histological aspects of each of these lesions. The treatment and the recurrence risk will also be discussed.

BACKGROUND: To minimize the risk of viscera exposure for parietal or calverial reconstruction after tumor removal, we used the two-stage free flap strategy. The flap was transferred a few days before tumor resection and left in a standby position until the second stage.
METHODS: We conducted a retrospective monocentric study. All patients who underwent reconstruction with the two-stage free flap strategy after tumor resection since 2000 were included.
RESULTS: We performed 14 two-stage flaps (8 for calvaria, 3 for abdomen, and 3 for thorax) on 12 patients. The average skin paddle surface was 318 cm2. The mean operative time was 274min for the first stage and 172min for the second stage. The average time between the two stages was 8.8 days (2 to 24 days). One flap necrosis, one venous thrombosis, and one hematoma were observed after the first stage. Partial skin paddle necrosis (2 flaps) and infections (3 flaps) occurred after the second stage. The mean follow-up was 20 months (6 to 61 months), and two patients had tumor recurrence.
CONCLUSIONS: The two-stage free flap strategy is another option for major oncological reconstructions, to be safe and reliable some rules must be followed. The flap must contain a large skin paddle to ensure flap autonomization and to allow for complete tight plication of the flap between the two stages, which limits germ colonization. A short delay between the two stages (<12 days) decreases the risk of infection. The presence of a plastic surgeon during the second stage decreases the risk of pedicle trauma.

From surviving fraction to tumour curability, definitions of tumour radioresistance may vary depending on the view angle. Yet, mechanisms of radioresistance have been identified and involve tumour-specific oncogenic signalling pathways, tumour metabolism and proliferation, tumour microenvironment/hypoxia, genomics. Correlations between tumour biology (histology) and imaging allow theragnostic approaches that use non-invasive biological imaging using tracer functionalization of tumour pathway biomarkers, imaging of hypoxia, etc. Modelling dose prescription function based on their tumour radio-resistant factor enhancement ratio, related to metabolism, proliferation, hypoxia is an area of investigation. Yet, the delivery of dose painting by numbers/voxel-based radiotherapy with low lineal energy transfer particles may be limited by the degree of modulation complexity needed to achieve the doses needed to counteract radioresistance. Higher lineal energy transfer particles or combinations of different particles, or combinations with drugs and devices such as done with radioenhancing nanoparticles may be promising.

The impact of curative radiotherapy depends mainly on the total dose delivered homogenously in the target volume. Tumor sensitivity to radiotherapy may be particularly inconstant depending on location, histology, somatic genetic parameters and the capacity of the immune system to infiltrate the tumor. In addition, the dose delivered to the surrounding healthy tissues may reduce the therapeutic ratio of many radiation treatments. In a same population treated in one center with the same technique, it appears that individual radiosensitivity clearly exists, namely in terms of late side effects that are in principle non-reversible. This review details the different radiobiological approaches that have been developed to better predict the tumor response but also the radiation-induced late effects.

OBJECTIVE: To provide a systematic review on ophthalmologic presentations of dermatofibrosarcoma protuberans (DFSP) in conjunction with a case report of primary orbital involvement by this tumor.
METHODS: A thorough electronic search on PubMed, Medline, Scopus, EMBASE and web of science databases was performed. All available data from reported cases along with our described case were extracted and analyzed.
RESULTS: We describe a 66-year-old male with primary orbital DFSP who was managed by total excision of the tumor without recurrence after 18 months of follow-up. In the literature, 14 studies reporting 15 cases were identified and reviewed. Our review topics included epidemiology, clinical presentations, pathologic features, differential diagnosis, imaging, treatment and prognosis.
CONCLUSIONS: Rarely, but crucially, ophthalmologists may encounter DFSP in the orbital or periorbital area. They should be familiar with the pathologic nature and treatment options for this tumor. This diagnosis should be kept in mind when approaching a case with a non-tender, nodular, firm, mass or more rarely an indenting orbital mass.

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Nodular fasciitis is a benign reactive lesion, often mistaken for a soft-tissue sarcoma in clinical practice. Involvement of the finger is rare, and a finger joint even rarer. We report on the clinical, radiological and histological features of intra-articular nodular fasciitis in a 52-year-old man, originating from the proximal interphalangeal joint of the right ring finger, with cortical erosion of adjacent bone. The discussion is focused on the tumor diagnosis and therapeutic approach, the differential diagnosis and the importance of immunohistochemical staining to establish the final diagnosis.

[This corrects the article DOI: 10.11604/pamj.2014.18.252.4684.].