Phosphaturic mesenchymal tumor: An underdiagnosed rare entity.

Tumor-induced osteomalacia is a paraneoplastic syndrome resulting in renal phosphate wasting and decreased bone mineralization. Phosphaturic mesenchymal tumors represent a rare etiology of tumor-induced osteomalacia. They are exceptionally rare, probably accounting for < 0.01% of all soft tissue tumors. Most PMTs present as small inapparent lesions that require very careful clinical examination and radionucleotide scan for localization. Here we describe a case in a 65 years old woman with recurrent multiple bone fractures and subsequent detection of a tumor involving right femur and adjacent soft tissue, low phosphate level and elevated serum Fibroblast growth factor-23 (FGF-23).

Carinal Resection and Reconstruction for an Obstructing Inflammatory Myofibroblastic Tumor in a Child.

Inflammatory myofibroblastic tumor (IMT) is a rare soft tissue tumor characterized by proliferation of fibroblastic cells associated with an inflammatory infiltrate. Inflammatory myofibroblastic tumors have a predilection for the pediatric population and are usually found in the lung parenchyma but rarely at the carina. They rarely metastasize but can be locally destructive. Surgical resection is the cornerstone of therapy, which results in excellent survival despite risk of local recurrence. We present the case of a nine-year-old girl with an IMT mass at the carina and obstructing the left main stem bronchus, requiring extensive resection and reconstruction.

Torsion of a mesenchymal hamartoma of the liver in a 1-year-old boy.

We report a case of a one-year-old boy who was referred to our clinic suspected of having acute abdomen. On physical examination, the abdomen was soft, diffusely tender with weak peristalsis. Ultrasonography and MRI of the right hemiabdomen demonstrated a well-defined, solid, expansive formation with slightly lobulated contours and an interspersed inhomogeneous structure with overall dimensions of 59 × 45 × 50 mm. After midline laparotomy was performed, a cystic tumor was found, twisted around a pedicle which was arising from the falciform ligament and it measured 5-6 cm in diameter. The tumor appeared to be necrotic. The mass was ligated and extirpated on the pedicle and sent for histopathological analysis. After the surgery, the boy was hemodynamically stable, without respiratory complications and all laboratory findings were within normal limits. Histopathological analysis showed that the tumor was composed of mesenchymal stroma with sparse glimpses of hepatocytes and bile ducts with partly cystic changes lined by orderly epithelium. Given the clinical data, histology and immunohistochemistry analysis (alpha-fetoprotein, CK8/18, hepatocyte, desmin and CD31) a diagnosis of a twisted mesenchymal hamartoma of the liver was made.

Embryologically based radical hysterectomy as peritoneal mesometrial resection (PMMR) with pelvic and para-aortic lymphadenectomy for loco-regional tumor control in endometrial cancer: first evidence for efficacy.

OBJECTIVE: To evaluate the feasibility and efficacy of embryologically based compartmental surgery for locoregional tumor control in intermediate and high risk endometrial cancer: peritoneal mesometrial resection with therapeutic pelvic and para-aortic lymphadenectomy by robotically assisted laparoscopy. METHODS: 75 consecutive surgically treated patients with uterine malignancies have been analyzed. 68 patients with histologically proven endometrial cancer and complete robotically assisted surgery have been included in this study on morbidity and oncological outcome. 56 % of the patients were at intermediate/high risk with either stage IAG3 or IB (n = 22) or stage II-IV (n = 16). Adjuvant EBRT was offered to three patients only (4 %), whereas five received isolated vaginal brachytherapy (7 %). Indocyanine-green (ICG) fluorescence lymphography is demonstrated being useful for additional intraoperative visualization of the compartment borders and lymphatic drainage to the postponed lymph compartments. RESULTS: After a mean follow-up of 32 months, there were only two loco-regional recurrences (2.9 %). Both recurrences were apparently cured by salvage therapy. 9 patients died; 6 (8.8 %) from metastatic disease (5) or unknown cause (1), 3 (4.4 %) from intercurrent disease without evidence of disease. One patient (1.4 %) experienced a peritoneal carcinosis and is alive. There were 8/68 perioperative complications (12 %). No perioperative mortality was observed. CONCLUSIONS: Embryologically defined compartmental surgery by robotically assisted laparoscopy seems to be feasible and safe in endometrial cancer. The low loco-regional recurrence rate of 2.9 % in spite of a very low percentage of adjuvant radiotherapy and 56 % of intermediate/high risk tumors should stimulate to initiate a multicentre trial to evaluate the value of compartmental surgery for prevention of locoregional recurrence in endometrial cancer.

Incidental gastric mesenchymal tumors identified during laparoscopic sleeve gastrectomy.

BACKGROUND: Laparoscopic sleeve gastrectomy (SG) is a well-tolerated and effective procedure for sustained weight loss and amelioration of weight-related co-morbidities. Rarely, unexpected pathology may be identified intraoperatively, which may alter the surgical plan. Gastrointestinal stromal tumors (GISTs) are among the more frequently encountered tumors and pose a particular concern because of their malignant potential. We review our findings of incidental tumors encountered during 1415 consecutive SGs. METHODS: Abnormal pathology records from all patients who underwent SG at our institution between 2009 and 2014 were reviewed. Patient demographic characteristics and clinical characteristics, tumor characteristics, including immunohistochemistry, operative course, and patient follow-up were reviewed. RESULTS: There were 17 incidental gastric mesenchymal tumors identified (1.2%) in 1415 SG procedures. This included 12 GISTs (.8%), 2 schwannomas (.1%), and 3 leiomyomas (.3%). In the majority of cases (1210/1415), the gastric specimens were not reviewed by a pathologist because there were no gross abnormalities appreciated by the surgeon. The GISTs were between .3 and 2.9 cm, and all were low grade with negative margins. Patients with GISTs tended to be older (mean age 55±9.3 y) than the rest of the patients. There was no evidence of recurrence on follow-up. CONCLUSION: Incidental gastric mesenchymal tumors are rarely encountered during SG. The vast majority were GISTs with an incidence of .8% in this population. Concomitant SG and tumor resection were feasible, without compromising the objectives of each. Complete tumor excision is necessary for tumors>2 cm.

Lipomatous variant of angiomyofibroblastoma: a case report and review of the literature.

Angiomyofibroblastoma represents a rare, benign mesenchymal tumor with a predilection for the vulvovaginal region. Lipomatous change may occur but rarely comprises a substantial component of the lesion. There are only eight reports in the English language literature describing the lipomatous variant of this tumor. We describe a further lipomatous angiomyofibroblastoma that occurred on the labium majus of a 49-year-old woman. The histopathologic and immunohistochemical features are described, and the collective experience in the literature is reviewed.

Leiomioma vascular en el pie: correlación ecográfica e histológica / Vascular leiomyoma of the foot: ultrasound and histologic correlation

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Juxta-articular myxoma: an unusual benign mesenchymal lesion, readily mistaken for malignancy.

Myxomas are benign tumours of mesenchymal origin. We describe the first reported case of paraspinal juxta-articular myxoma. Juxta-articular myxomas show increased cellularity and distinction from cellular myxoma is required. The differential also includes malignant myxofibrosarcoma. For patient prognosis and management it is essential to separate these entities. Complete surgical excision is the mainstay of treatment as local recurrences may occur.

Primary mesenchymal tumors of the pancreas: single-center experience over 16 years.

OBJECTIVE: Primary mesenchymal tumors of the pancreas are extremely rare and no comprehensive study of this class of tumors has been previously performed. METHODS: Twenty cases of the primary mesenchymal tumors of the pancreas were selected and compared clinicopathologic variables with consecutively resected 500 pancreatic ductal adenocarcinomas. RESULTS: Finally, 20 (0.3%) cases were confirmed as primary mesenchymal tumors. The 14 (70%) cases of benign/borderline tumors included 4 cases of fibromatoses, 2 cases of cavernous hemangiomas, 2 cases of schwannomas, 2 cases of solid and cystic hamartomas, 2 cases of solitary fibrous tumors, 1 case of inflammatory myofibroblastic tumor, and 1 case of angiomyolipoma. The 6 (30%) cases of sarcomas comprised 3 cases of undifferentiated/unclassified sarcomas, 1 case of leiomyosarcoma, 1 case of Ewing sarcoma/primitive neuroectodermal tumor, and 1 case of atypical lipomatous tumor/well-differentiated liposarcoma. When compared with the 500 ductal adenocarcinomas, the 3 surgically resected sarcomas were larger (mean, 5.8 cm vs 3.6 cm; P = 0.02); however, no difference in median survival time was observed between patients with sarcoma (23 months) and patients with ductal adenocarcinoma (16 months). CONCLUSIONS: First, primary mesenchymal tumors of the pancreas are extremely rare. Second, several primary mesenchymal tumor types can be observed in the pancreas. Understanding these rare disease entities will help ensure their correct diagnosis.

Giant dermatofibrosarcoma protuberans - rare form of mesenchymal tissue neoplasm: case presentation.

UNLABELLED: Dermatofibrosarcoma protuberans (DFSP), a rare type of mesenchymal neoplasm, is defined by the WHO as a superficial sarcoma with low-grade malignancy that develops in the cutaneous and subcutaneous tissues. The purpose of this paper is to present a case of a giant DFSP, with post-traumatic onset in childhood and a very long evolution. CLINICAL DATA: 51-year-old Caucasian patient presents for 41 years a presternal neoplastic lesion, with onset at 10-year-old, few months after a strong trauma. The patient addressed for a clinic examination, secondary to a spontaneous hemorrhage of the lesion. The local examination reveals the presence of a red-purple polynodular neoplastic lesion of 180×110×30 mm, of firm consistency, adherent to the subcutaneous tissue, painless, with lateral extension at 8 o'clock as an erythematous infiltrated atrophic plaque appearance. One of these nodular masses presents surface ulceration and areas of necrosis. The CT scan did not detect any infiltration into the pectoral muscle or loco-regional metastasis. Under general anesthesia a wide surgical excision with free macroscopic margins of 3 cm was performed. Histopathological diagnosis was DFSP, with evidence of tumoral spindle cells disposed in storiform pattern, embedding small adipocyte panicles, creating a lace-like or honeycomb appearance. Immunohistochemically, the tumor cells express an intense and diffuse CD34 and they are negative for S-100 and SMA. The Ki-67 is focal positive in almost 2-4%. Clinical and paraclinical monitoring at 18 months follow-up does not detect any local recurrences or metastases, and an excellent quality of life.

Tumoraciones mesenquimales de músculo liso uterino de potencial incierto: revisión anatomopatológica y pronóstica de un caso clínico / Uterine smooth-muscle tumors of uncertain malignant potential: histopathological findings and outcome in a clinical case

Los STUMPs (tumoraciones mesenquimales de músculo liso uterino de potencial incierto) son tumoraciones infrecuentes, que pertenecen a las tumoraciones mesenquimales de músculo liso uterino. Estas tumoraciones no pueden definirse ni como totalmente benignas ni malignas. Presentamos un caso clínico de una paciente de 27 años que consultó por sensación de plenitud, aumento del perímetro abdominal y polaquiuria de 6 meses de evolución. Ecográficamente se visualizó una masa intramural-subserosa de 9 cm en fondo uterino, con sospecha de degeneración hialina, que fue extirpada quirúrgicamente. El estudio anatomopatológico reveló el diagnóstico de tumoración muscular lisa de potencial maligno incierto, variante epiteloide. Conclusión. Los STUMPs se diagnostican anatomopatológicamente, siendo este proceso complejo y requiriendo, en gran número de ocasiones, técnicas inmunohistoquímicas para conseguir un diagnóstico y pronóstico más preciso. El tratamiento es quirúrgico, pudiendo realizar una histerectomía o bien, siendo más conservadores, la exéresis de la tumoración, ya que la mayoría tienen un comportamiento benigno (AU)

Uterine smooth-muscle tumours with unusual growth patterns represent an histologically heterogeneous and uncommon group of uterine smooth-muscle tumours that cannot be diagnosed as either benign or malignant.We report the case of a 27year-old woman who consulted for a 6-month history of bladder fullness sensation, abdominal distension and polaquiuria. Transvaginal ultrasound showed a 9cm intramural mass at the uterine fundus that was surgically excised. The anatomopathological diagnosis of uterine smooth-muscle tumour with growth pattern, epithelioid variant, was reported. Conclusions: The diagnosis of uterine smooth-muscle tumours may create great challenges for the pathologist who classifies these tumours by their histological features. The immunohistochemical staining may be helpful to establish their behaviour and prognosis. The clinical management remains a dilema and surgical treatment of choice has not been well defined, due to their uncertain clinical behaviour. Most of these tumours have a benign clinical course but these patients should receive long-term follow-up (AU)

Lipoma gigante de Membro inferior com repercussão no sistema vascular / Giant lipoma of the lower limb and its impact on the vascular system

Lipomas are the most common mesenchymal tumors, which may develop in different parts of the body. However giant lipomas are rare and sometimes difflcult to diagnose owing to their location and the presenting clinical consequences. In this report, we present the case of a patient with a giant lipoma in lhe left popliteal region that was surgícally treated after a detailed radiologícal assessment. Further, we discuss lhe preoperative diagnosis and its difficulties, often caused by the deep localization of the tumor and the impossibility of obtaining a conclusive radiological diagnosis. Moreover, we describe the successful treatment, along with the step-by-step surgical approach and the final outcome achieved.

Os lipomas são os tumores Mesenquimais mais frequentes e podem ocorrer em diversas partes do corpo. Entretanto lipomas gigantes são raros por vezes de difícil diagnóstico, em decorrência de sua localização e com consequência clinicas. Neste artigo é apresentado um caso de lipoma gigante em região poplítea esquerda tratado cirurgicamente após detalhado estudo radiológico. São discutidos o diagnóstico pré-operatório e suas dificuldades, muitas vezes causadas pela localização profunda e pela impossibilidade de diagnóstico conclusivo pela imagem. O tratamento foi concluído com sucesso, sendo descritos a abordagem cirúrgica passo a passo e o resultado final.

Definition of compartment-based radical surgery in uterine cancer: radical hysterectomy in cervical cancer as 'total mesometrial resection (TMMR)' by M Höckel translated to robotic surgery (rTMMR).

BACKGROUND: Radical hysterectomy has been developed as a standard treatment in Stage I and II cervical cancers with and without adjuvant therapy. However, there have been several attempts to standardize the technique of radical hysterectomy required for different tumor extension with variable success. Total mesometrial resection as ontogenetic compartment-based oncologic surgery - developed by open surgery - can be standardized identically for all patients with locally defined tumors. It appears to be promising for patients in terms of radicalness as well as complication rates. Robotic surgery may additionally reduce morbidity compared to open surgery. We describe robotically assisted total mesometrial resection (rTMMR) step by step in cervical cancer and present feasibility data from 26 patients. METHODS: Patients (n = 26) with the diagnosis of cervical cancer were included. Patients were treated by robotic total mesometrial resection (rTMMR) and pelvic or pelvic/periaortic robotic therapeutic lymphadenectomy (rtLNE) for FIGO stage IA-IIB cervical cancer. RESULTS: No transition to open surgery was necessary. No intraoperative complications were noted. The postoperative complication rate was 23%. Within follow-up time (mean: 18 months) we noted one distant but no locoregional recurrence of cervical cancer. There were no deaths from cervical cancer during the observation period. CONCLUSIONS: We conclude that rTMMR and rtLNE is a feasible and safe technique for the treatment of compartment-defined cervical cancer.

Definition of compartment-based radical surgery in uterine cancer: modified radical hysterectomy in intermediate/high-risk endometrial cancer using peritoneal mesometrial resection (PMMR) by M Höckel translated to robotic surgery.

BACKGROUND: The technique of compartment-based radical hysterectomy was originally described by M Höckel as total mesometrial resection (TMMR) for standard treatment of stage I and II cervical cancer. However, with regard to the ontogenetically-defined compartments of tumor development (Müllerian) and lymph drainage (Müllerian and mesonephric), compartments at risk may also be defined consistently in endometrial cancer. This is the first report in the literature on the compartment-based surgical approach to endometrial cancer. Peritoneal mesometrial resection (PMMR) with therapeutic lymphadenectomy (tLNE) as an ontogenetic, compartment-based oncologic surgery could be beneficial for patients in terms of surgical radicalness as well as complication rates; it can be standardized for compartment-confined tumors. Supported by M Höckel, PMMR was translated to robotic surgery (rPMMR) and described step-by-step in comparison to robotic TMMR (rTMMR). METHODS: Patients (n = 42) were treated by rPMMR (n = 39) or extrafascial simple hysterectomy (n = 3) with/without bilateral pelvic and/or periaortic robotic therapeutic lymphadenectomy (rtLNE) for stage I to III endometrial cancer, according to International Federation of Gynecology and Obstetrics (FIGO) classification. Tumors were classified as intermediate/high-risk in 22 out of 40 patients (55%) and low-risk in 18 out of 40 patients (45%), and two patients showed other uterine malignancies. In 11 patients, no adjuvant external radiotherapy was performed, but chemotherapy was applied. RESULTS: No transition to open surgery was necessary. There were no intraoperative complications. The postoperative complication rate was 12% with venous thromboses, (n = 2), infected pelvic lymph cyst (n = 1), transient aphasia (n = 1) and transient dysfunction of micturition (n = 1). The mean difference in perioperative hemoglobin concentrations was 2.4 g/dL (± 1.2 g/dL) and one patient (2.4%) required transfusion. During follow-up (median 17 months), one patient experienced distant recurrence and one patient distant/regional recurrence of endometrial cancer (4.8%), but none developed isolated locoregional recurrence. There were two deaths from endometrial cancer during the observation period (4.8%). CONCLUSIONS: We conclude that rPMMR and rtLNE are feasible and safe with regard to perioperative morbidity, thus, it seems promising for the treatment of intermediate/high-risk endometrial cancer in terms of surgical radicalness and complication rates. This could be particularly beneficial for morbidly obese and seriously ill patients.

Separation of mouse embryonic facial ectoderm and mesenchyme.

Orofacial clefts are the most frequent craniofacial defects, which affect 1.5 in 1,000 newborns worldwide. Orofacial clefting is caused by abnormal facial development. In human and mouse, initial growth and patterning of the face relies on several small buds of tissue, the facial prominences. The face is derived from six main prominences: paired frontal nasal processes (FNP), maxillary prominences (MxP) and mandibular prominences (MdP). These prominences consist of swellings of mesenchyme that are encased in an overlying epithelium. Studies in multiple species have shown that signaling crosstalk between facial ectoderm and mesenchyme is critical for shaping the face. Yet, mechanistic details concerning the genes involved in these signaling relays are lacking. One way to gain a comprehensive understanding of gene expression, transcription factor binding, and chromatin marks associated with the developing facial ectoderm and mesenchyme is to isolate and characterize the separated tissue compartments. Here we present a method for separating facial ectoderm and mesenchyme at embryonic day (E) 10.5, a critical developmental stage in mouse facial formation that precedes fusion of the prominences. Our method is adapted from the approach we have previously used for dissecting facial prominences. In this earlier study we had employed inbred C57BL/6 mice as this strain has become a standard for genetics, genomics and facial morphology. Here, though, due to the more limited quantities of tissue available, we have utilized the outbred CD-1 strain that is cheaper to purchase, more robust for husbandry, and tending to produce more embryos (12-18) per litter than any inbred mouse strain. Following embryo isolation, neutral protease Dispase II was used to treat the whole embryo. Then, the facial prominences were dissected out, and the facial ectoderm was separated from the mesenchyme. This method keeps both the facial ectoderm and mesenchyme intact. The samples obtained using this methodology can be used for techniques including protein detection, chromatin immunoprecipitation (ChIP) assay, microarray studies, and RNA-seq.

Double-balloon enteroscopy for mesenchymal tumors of small bowel: nine years' experience.

AIM: To assess the value of double-balloon enteroscopy (DBE) for the diagnosis of gastrointestinal mesenchymal tumors (GIMTs) in the small bowel and clarify their clinical and endoscopic characteristics. METHODS: A retrospective review in a total of 783 patients who underwent a DBE procedure from January 2003 to December 2011 was conducted. Data from patients with pathologically confirmed GIMTs were analyzed at a single tertiary center with nine years' experience. The primary outcomes assessed included characteristics of patients with GIMTs, indications for DBE, overall diagnostic yield of GIMTs, endoscopic morphology, positive biopsy, comparison of diagnosis with capsule endoscopy, and subsequent interventional management. RESULTS: GIMTs were identified and analyzed in 77 patients. The mean age was 47.74 ± 14.14 years (range: 20-77 years), with 63.6% being males. The majority of individuals presented with gastrointestinal bleeding, accounting for 81.8%, followed by abdominal pain, accounting for 10.4%. Small bowel pathologies were found in 71 patients, the detection rate was 92.2%. The diagnostic yield of DBE for GIMTs was 88.3%. DBE was superior to capsule endoscopy in the diagnosis of GIMTs (P = 0.006; McNemar's χ(2) test). Gastrointestinal stromal tumor was the most frequent and leiomyoma was the second frequent GIMT. Single and focal lesions were typical of GIMTs, and masses with smooth or unsmooth surface were the most common in the small bowel. GIMTs were removed from all the patients surgically except one patient treated with endoscopic resection. CONCLUSION: DBE is a safe and valuable procedure for patients with suspected GIMTs, and it provides an accurate position for subsequent surgical intervention.

Prenatal and postnatal features of mesenchymal hamartoma of the chest wall: case report and literature review.

Mesenchymal hamartoma of the chest wall is a rare, benign chondro-osseous tumor of the bone. Although it most commonly presents at birth or soon after, prenatal detection is rare. We report a case of prenatally detected mesenchymal hamartoma, and provide the rationale, details, and outcomes of our management. The literature is reviewed, with particular attention to prenatal detection and postnatal management options.