Nasal chondromesenchymal hamartoma (NCMH) is an extremely rare benign tumor, primarily diagnosed in young infants and children and it often simulates malignant tumors on imaging.
We present computerized tomography and magnetic resonance imaging findings of two nasal chondromesenchymal hamartomas in a 5-year-old boy and a 6-week-old girl.
NCMH is a rare, benign tumor-like lesion with good biologic behavior. No recurrence after complete resection or malignant transformation of NCMH has been reported. A correct diagnosis is imperative to avoid unnecessary adjuvant therapy.
Computerized tomography; Magnetic resonance imaging; Nasal chondromesenchymal hamartoma
Nasal chondromesenchymal hamartoma (NCMH) is a rare nasal tumor that typically presents in young children. We previously reported on NCMH occurrence in children with pleuropulmonary blastoma (PPB), a rare pulmonary dysembryonic sarcoma that is the hallmark neoplasm in the PPB-associated DICER1 tumor predisposition disorder.
Original pathologic materials from individuals with a PPB, PPB-associated tumor and/or a DICER1 mutation were centrally reviewed by the International PPB Registry. Paraffin-embedded NCMH tumor tissue was available in three cases. Laser-capture microdissection was used to isolate mesenchymal spindle cells and cartilage in one case for Sanger sequencing of DICER1.
Nine patients (5F/4M) had PPB and NCMH. NCMH was diagnosed at a median age of 10 years (range 6-21years). NCMH developed 4.5 - 13 years after PPB. Presenting NCMH symptoms included chronic sinusitis and nasal congestion. Five patients had bilateral tumors. Local NCMH recurrences required several surgical resections in two patients, but all nine patients were alive at 0 – 16 years of follow-up. Pathogenic germline DICER1 mutations were found in 6/8 NCMH patients tested. In 2 of the patients with germline DICER1 mutations, somatic DICER1 missense mutations were also identified in their NCMH (E1813D; n=2). Three additional PPB patients developed other nasal lesions seen in the general population (a Schneiderian papilloma, chronic sinusitis with cysts, and allergic nasal polyps with eosinophils). Two of these patients had germline DICER1 mutations.
Pathogenic germline and somatic mutations of DICER1 in NCMH establishes that the genetic etiology of NCMH is similar to PPB, despite the disparate biological potential of these neoplasms.
Nasal chondromesenchymal hamartoma; DICER1; Pleuropulmonary blastoma
Breast hamartomas are rare, benign, tumor-like nodules composed of glandular, adipose and fibrous tissue. The hamartoma was first described in 1971 as a lipofibroadenoma, fibroadenolipoma or adenolipoma, based on the predominant component of the breast tissue. Clinical findings resemble fibroadenoma and if there is a palpable mass, the patients may receive an immediate diagnosis. Ductal hyperplasia, apocrine metaplasia, calcification and adenosis may occur within the hamartoma, with rarer instances of lobular or ductal intraepithelial neoplasms. Although hamartoma is usually benign, a malignant transformation is possible. An excision and histological examination is necessary for the differential diagnosis and also for any epithelial lesions of the hamartoma. Simple excision is enough for treatment if there is no coincidental epithelial malignant lesion. The patients in the present study were treated by simple excision as there were no proliferative changes in the lesions. No recurrence or other problems were detected in the 18-month follow-up. The current study presents two cases of breast hamartoma that were diagnosed as an adenolipoma and a fibroadenolipoma, and then describes the macroscopic and microscopic observations of these lesions.
hamartoma; breast; adenolipoma
Pancreatic tumor is a rare condition in children, but reasonably common in adults. Histopathology in children also differs from that in adults, with most cases being pancreatoblastoma, solid pseudopapillary tumor, or pancreatic endocrine tumors.
PRESENTATION OF CASE
A 14-month-old boy was noticed abdominal distension and referred to our hospital. Laboratory findings revealed leukocytosis and elevation of serum level of C-reactive protein and pancreatic enzymes. Radiological findings at admission were the huge abdominal cyst in abdominal computed tomography. As the levels of pancreatic enzyme elevated synchronous to oral feeding, total parenteral nutrition was needed. Besides radiographically the abdominal lesion changed from multiple large cystic type to multiple microcystic lesion including solid component over time. It was considered different diagnosis was pancreatic blastoma, pancreatic pseudo cyst, and lymphangioma and he was performed operation. The huge multicystic and partially solid tumor arising from the tail of pancreas existed from posterior of stomach to pelvic cavity. The tumor was completely resected without pancreatectomy and residual pancreas can be kept without tumor invasion. Histopathological finding was pancreatic hamartoma.
Pancreatic hamartoma was extremely rare, and only 17 cases were previously reported in the literature. This is the first case that the change of radiographic findings overtime was shown. We reviewed 17 cases (4 cases in children) of pancreatic hamartoma including our case.
Although extremely rare, pancreatic hamartoma should be considered in the differential diagnosis of cystic abdominal mass in children.
Pancreas; Hamartoma; Pancreatitis; Child
Mammary hamartomas are typically a benign condition and rarely develop into malignant lesions. Only 14 cases of carcinomas associated with a hamartoma have been documented in the literature. In this case report, we describe a case of invasive ductal carcinoma within a hamartoma in a 72-year-old woman. Mammography, ultrasonography, and magnetic resonance imaging showed the features of a typical hamartoma with a suspicious mass arising in it. This case illustrates the importance of identification of unusual findings in a typical mammary hamartoma on radiologic examinations.
Mammary hamartoma; Invasive ductal carcinoma
A pancreatic hamartoma is a rare benign lesion that may be mistaken for malignancy. A pancreatic hamartoma can present with vague, non-specific symptoms, which can be difficult to diagnose despite modern diagnostic tools. We report here a pancreatic hamartoma diagnosed after surgical resection. A 52-year-old female presented with postprandial abdominal discomfort. Abdominal computed tomography and pancreatic magnetic resonance imaging revealed a 2.2 × 2.5-cm cystic mass in the pancreatic head. The patient underwent a pylorus-preserving pancreaticoduodenectomy. The histopathological and immunohistochemical studies helped make the diagnosis of pancreatic hamartoma. Here, we report a case of pancreatic hamartoma and review the relevant medical literature.
Hamartoma; Pancreas; Pancreaticoduodenectomy
A myoepithelial hamartoma is a very uncommon submucosal tumor of the stomach. In an atypical presentation in our case, it mimicked the clinical presentation of a gastrointestinal stromal tumor. To the best of our knowledge, it is the first case of a hamartoma of the stomach reported from Bangladesh and one of few cases described in the literature.
We describe the case of a 35-year-old Bengali man with recurrent epigastric pain and occasional vomiting with radiographic findings of a gut mass. An upper gastrointestinal endoscopy revealed a healed duodenal ulcer, deformed ‘D’ bulb and a submucosal swelling in his antrum. Ultrasonography and a contrast-enhanced computed tomography scan confirmed the presence of a well-defined, oval gut mass in his upper abdomen, compressing his duodenum. The mass had a mixed density and was considered to probably be a gastrointestinal stromal tumor. Ultrasonography-guided fine needle aspiration cytology was inconclusive. After resection at laparotomy, a histopathological examination revealed a myoepithelial hamartoma. These tumors are characterized by hypertrophic smooth muscle bands surrounding varied epithelial elements, which may be arranged in diverse patterns such as simple glandular structure, Brunner’s gland, pancreatic ducts and sometimes pancreatic acini. This case report is complemented by a literature review relating to the atypical presentation.
Gut masses need to be investigated thoroughly and the possibility of rare tumors should not be excluded. Although the recommended treatment for such lesions is limited resection, radical procedures such as a pancreaticoduodenectomy are often performed when the lesion occurs in the periampullary area because of preoperative misdiagnosis as a carcinoma. Therefore, it is essential for clinicians to maintain current knowledge of the lesion to avoid inaccurate diagnosis and prevent unnecessary surgery.
The sinonasal tract is a complex anatomic site, home to a wide variety of reactive, inflammatory, benign, and malignant lesions. Inflammatory polyps and papillomas are usually easily recognized by pathologists. A poorly understood lesion that has been more clearly defined in recent years is the nasal hamartoma. The epithelial subtypes include seromucinous hamartoma, respiratory epithelial adenomatoid hamartoma, and hybrid lesions. Seromucinous hamartomas have only been recognized and substantially reported over the past few years. They are a diagnostic challenge, needing to be distinguished from low grade adenocarcinomas, and are of interest because most of the basic questions about their pathophysiology remain unanswered. Herein, we present two novel cases of seromucinous hamartoma with features that partly expand the morphologic spectrum of these lesions, discuss the differential diagnosis, and review the literature to compare our findings with previously reported cases with the aim of better understanding this interesting entity.
Seromucinous hamartoma; Microglandular adenosis; Low grade adenocarcinoma; Nasal cavity; Respiratory epithelial adenomatoid hamartoma
Fibroma, neurofibroma, and papilloma are the most commonly encountered benign lesions in the nasopharynx. Hamartomas are non-neoplastic overgrowth of mature/differentiated tissue indigenous to the specific part of the body in which they develop. Most hamartomas are located in the liver, spleen, lungs, and pancreas. However, nasopharyngeal hamartoma is rare.
We describe here a 77-year-old Japanese woman who presented with a mass arising from the left lateral wall of the nasopharynx. Computed tomography (CT) revealed a soft tissue mass without bony erosion, suggesting that the mass was a benign tumor such as a fibroma. Pathological examination showed that the mass was a leiomyomatous hamartoma.
To our knowledge, this is the first report of a leiomyomatous hamartoma in the nasopharynx. Although leiomyomatous hamartoma in the nasopharynx is extremely rare, it should be kept in mind during differential diagnosis.
Hamartoma; Nasopharynx; Otolaryngologic tumor
Hamartomas are non-neoplastic lesions constituted by a mixture of tissues indigenous to the region. Respiratory epithelial adenomatoid hamartomas are characterised by glandular proliferation lined by ciliated airway epithelium. Their localisation in the nasal cavity is rare and most frequent cases described so far were associated with the posterior nasal septum.
A 9-year-old Caucasian boy presented with long-standing nasal obstruction. A large right nasal mass was evident on physical and CT examinations. It was surgically removed from the anterior nasal septum under general anaesthesia. Histologically, the diagnosis of REAH was established. The tumour lined by stratified squamous and ciliated respiratory epithelium was characterised by prominent glandular proliferation. By immunohistochemistry, the tumour was positive for cytokeratins, smooth muscle actin, vimentin, laminin, collagen type IV, CD8, and CD68. No S-100 immunoreactivity was observed. The patient has been asymptomatic for 12 months with completely healed lining of the nose.
Respiratory epithelial adenomatoid hamartoma, although rare, must be taken into consideration in differential diagnosis of nasal exophytic lesions.
Splenic hamartoma is a rare benign tumor, and although minimally invasive surgery may be suitable for this condition, there have only been 2 previous reports of laparoscopic surgery. Here we report the third case of splenic hamartoma managed by laparoscopic splenectomy. A 37-year-old male was incidentally diagnosed by abdominal ultrasonography with a hypoechoic mass measuring 2.5 cm × 2.4 cm in the spleen. Color Doppler sonography showed multiple flow signals within the mass and contrast-enhanced computed tomography revealed strong enhancement of the lesion. On T1- and T2-weighted magnetic resonance images, the splenic mass was demonstrated as isointense and hyperintense respectively. Although a malignant tumor could not be ruled out, a hand-assisted laparoscopic splenectomy was performed because the splenic mass was limited in size and had not invaded adjacent organs. The pathological diagnosis was splenic hamartoma. The postoperative course was uneventful and the patient was discharged by the seventh postoperative day. Although splenic hamartomas have some specific imaging features, more reports and analyses of these cases are required to increase the reliability of the diagnosis and management. Hand-assisted laparoscopic splenectomy may play a pivotal role in the postoperative diagnosis and management of this condition.
Splenic hamartoma; Splenoma; Splenic tumor; Laparoscopic splenectomy; Splenectomy
Pulmonary hamartoma is a benign tumor composed of cells and tissues, amorphous demarcated. It occurs in 0.25% of the general population (in autopsy material) and in 6-8% of all benign solitary lung nodules.
Patients and methods
A male patient, smoker 30 p/y, reports a month weakness, fatigue, and low grade fever without weight loss. The XR of the chest showed a solid formation mass with fuzzy borders in contact with the left lung hilum. The chest CT revealed a lesion 5.5 cm × 4.5 cm in the anterior segment of the left upper lobe and little enlargement of lymph nodes (superior mediastina, of aortopulmonary window and right paratracheal). Therefore, the patient underwent diagnostic bronchoscopy.
The endoscopic image showed an exophytic lesion occludes one of the subsegment bronchi of the anterior of the left upper lobe with smooth, polished, pale yellow surface coalesced into a broad basis to the underlying bronchi. The difficulty in obtaining the biopsy material by the forcep (due to hardness) confirmed the histopathological examination which revealed particles of tissue, composed of adipose tissue, cartilage elements, sparse bone trabeculae, hematopoietic marrow elements and fibrin myxomatoid substrate, a typical pulmonary hamartoma. The patient underwent thoracic MRI in order to exclude a possible coexisting lung cancer and for the evaluation of mediastinal lymph nodes, head, abdominal and bone CT scan (without pathological findings). It is expected the scheduling of surgical removal of the lesion as a definitive treatment for the patient.
Pulmonary hamartoma occurs most often in 5th-6th decade of life with a ratio males: females 3:1. Only in 10% is intrabronchial (mainly in the periphery of lung parenchyma) causing obstruction and rarely can have multiple localization (Cowden syndrome). It is usually an incidental finding on chest XR and appears with cough, fever, pain, or rarely hemoptysis. The diameter varies from a few mm up to 4 cm (average 2.5 cm, remarkable is the size of the lesion in our case). It doesn’t format a pulmonary cavity while in 15% occurs central amorphous, almost pathognomonic, pop corn type calcifications. It grows slowly, about 5 mm per year. In a typical XR appears as a mass with clear boundaries and smooth edge and the characteristic (if it exists) calcifications, while CT scan detects the fat, the boundaries of the mass and the type of calcifications.
Seromucinous hamartoma is a benign lesion of the sinonasal tract. Since its description in 1974, only a small number of additional cases have been reported. It is composed of a proliferation of seromucinous glands and ducts within a variable fibrous stroma. The serous component typically stains positively for S100 (at least focally) and lacks p63 positive abluminal cells. The lack of myoepithelial/basal cells is an important diagnostic feature of seromucinous hamartoma; their absence could lead to an incorrect diagnosis of low-grade sinonasal adenocarcinoma. We report the case of a polypoid mass resected from the posterior nasal cavity and nasopharynx of a 54-year-old woman. The lesion contained a population of small and large glands lined by cuboidal to flattened cells within a hypocellular stroma varying from dense and sclerotic to myxoid. Additionally, there was a superficial focus of ciliated invaginated surface epithelium and glands. Throughout the lesion there were no cytologic or architectural features of malignancy. The histologic features were diagnostic of seromucinous hamartoma. Immunohistochemistry showed focal S100 positivity of the serous glands. However, in contrast to previously reported cases, the glands focally showed an outer basal layer that was calponin, p63 and actin positive. Our case demonstrates two important points. First, complete absence of p63 staining should not necessarily be a required feature in the diagnosis of seromucinous hamartoma. Second, the ciliated larger glands—in keeping with respiratory epithelial adenomatoid hamartoma (REAH)—support the suggestion that seromucinous hamartoma and REAH are a spectrum of lesions, often seen together.
Seromucinous hamartoma; Respiratory epithelial adenomatoid hamartoma; Low-grade sinonasal adenocarcinoma; Nasal tumors
Introduction. Lung hamartomas are the most common benign tumors of the lung. Typically,
they are located in the peripheral lung, while an endobronchial localisation is rare. Case Presentation. We present a case with the rare diagnosis of an endobronchial hamartoma as incidental finding in a 69-year-old male, caucasian patient with atrial
fibrillation. At first admission, the patient's exertional dyspnea was caused by atrial fibrillation. Relapse of exertional dyspnea in the absence of arrhythmia was due to postobstructive pneumonia caused by an endobronchial hamartoma. Conclusion. Endobronchial tumors such as endobronchial lipoma or hamartoma should be considered as potential causes of exertional dyspnea and thus as differential diagnosis of
atrial fibrillation. Although endobronchial hamartomas are benign, resection is recommended to prevent postobstructive lung damage.
Myoid hamartoma is an uncommon type of breast hamartoma and its recurrence is very rare. We report the imaging appearance of an unusual case of recurrent myoid hamartoma of the breast mimicking malignancy in a 43-year-old woman. Although the mammographic and ultrasonographic findings have long been described in the literature, MR finding with a dynamic study has not, to the best of our knowledge, been reported previously.
Breast; Myoid hamartoma; Hamartoma, MR
Non-neoplastic tumor-like lesions in the pancreas are uncommon. Here, we present a case of multiple solid pancreatic hamartomas in a 78-year-old Japanese woman. Her computed tomography revealed a pancreatic mass, measuring 1.8 cm in maximum diameter. However, no symptoms were found. She was not an alcoholic and had no history of pancreatitis. The patient underwent a pancreatoduodenectomy, and three well-demarcated solid nodules measuring 1.7 cm, 0.4 cm, and 0.3 cm in diameter were found in the pancreatic head. Microscopically, the lesions were composed of non-neoplastic, disarranged acinar cells and ducts embedded in a sclerotic stroma with elongated spindle cells that lacked discrete islets. The stromal spindle cells were immunoreactive for CD34 and CD117. The histological diagnosis was multiple solid hamartomas of the pancreas. There has been no recurrence 30 mo after surgery. So far, 18 cases of pancreatic hamartoma have been reported in the English literature, including our case. Six out of these 18 cases seemed to fit the criteria of solid pancreatic hamartoma. Although the number of cases was limited, solid pancreatic hamartomas seem to be benign tumor-like lesions, which are found incidentally in healthy middle-aged adults, but occasionally involve the whole pancreas, resulting in a poor prognosis. Solid pancreatic hamartoma was sometimes associated with minor pancreatic abnormality, and multiple small lesions other than the main tumors were detected in a small number of cases. From these findings, one may speculate that solid pancreatic hamartoma could be the result of a malformation during the development of the pancreas.
Pancreatic tumor; Hamartoma; Multiple; CD117; CD34
Germline mutations in DICER1 are associated with increased risk for a wide variety of neoplastic conditions, including pleuropulmonary blastoma (PPB), cystic nephroma, nasal chondromesenchymal hamartoma, ovarian Sertoli-Leydig cell tumors, botryoid embryonal rhabdomyosarcoma of the uterine cervix, ciliary body medulloepithelioma, pineoblastoma, pituitary blastoma and nodular thyroid hyperplasia or thyroid carcinoma. These tumors may be seen in isolation or in constellation with other characteristic tumor types in individuals or family members. Here we describe the medical history of a child with a heterozygous, loss of function germline DICER1 mutation and multiple tumors associated with the syndrome.. Although germline mutations in DICER1 are rare, tumors of these types will be seen by practicing pathologists and should prompt consideration of an underlying DICER1 mutation.
DICER1; pleuropulmonary blastoma; Sertoli-Leydig; thyroid carcinoma; nasal chondromesenchymal hamartoma
Aims: This study is to compare the value of ultrasonography, computed tomography (CT) and magnetic resonance imaging (MRI) in the diagnosis of biliary hamartomas. Methods: From 2003 to 2013, 15 cases of liver biopsies were found to have biliary hamartomas, including 3 cases excluded from this study. The remaining 12 patients were 7 women and 5 men aged from 28 to 66 years (mean age, 53 years). Ultrasonography examinations were performed by two different scanners using 3.5- to 5.0-MHz convex array transducers. Eight patients were examined by plain and contrast CT including 2 cases with Sensation Cardiac 64 and 6 cases with Somatom definition dual source CT. MRI was performed by a 3 T system using an eight-channel phased-array torso coil. Using pathology slides, lesions were classified into class 1 (predominantly solid), class 2 (intermediate, mixed solid and cystic), and class 3 (predominantly cystic). Results: Patients with biliary hamartomas have distributed lesions. Ultrasonography can be used to diagnose biliary hamartomas, with occasional mistakes. CT is effective in the diagnosis of biliary hamartomas. MRI is capable of diagnosing biliary hamartomas. Histopathological examination provides a direct means to classify the degrees of lesions caused by biliary hamartomas. Conclusions: Biliary hamartomas showed characteristic features on imaging findings by ultrasonography, CT, and MRI. Although abdominal ultrasonography could detect suspected biliary hamartomas, the best choice for further diagnosis is MRI examination instead of CT. In addition, follow-up ultrasonography examinations are necessary.
Biliary hamartoms; ultrasonography; computed tomography; magnetic resonance imaging
Breast hamartoma is an uncommon breast tumor that accounts for approximately 4.8% of all benign breast masses. The pathogenesis is still poorly understood and breast hamartoma is not a well-known disorder, so its diagnosis is underestimated by clinicians and pathologists. This study was designed to present our experience with breast hamartoma, along with a literature review.
We reviewed the demographic data, pathologic analyses and imaging and results of patients diagnosed with breast hamartoma between December 2003 and September 2013.
In total, 27 cases of breast hamartoma operated in the Ankara University Medicine Faculty's Department of General Surgery were included in the study. All patients were female and the mean age was 41.8±10.8 years. The mean tumor size was 3.9±2.7 cm. Breast ultrasound was performed on all patients before surgery. The most common additional lesion was epithelial hyperplasia (22.2%). Furthermore, lobular carcinoma in situ was identified in one case and invasive ductal carcinoma was observed in another case. Immunohistochemical staining revealed myoid hamartoma in one case (3.7%).
Breast hamartomas are rare benign lesions that may be underdiagnosed because of the categorization of hamartomas as fibroadenomas by pathologists. Pathologic examinations can show variability from one case to another. Thus, the true incidence may be higher than the literature indicates.
Breast; Hamartoma; Carcinoma
Adenoma is the most common cause of duodenal polyps, while hamartomas are very rare. We present a patient with a preoperative histology proved diagnosis of isolated duodenal tubulovillous adenomatous polyp with high-grade dysplasia for whom we performed laparoscopic antrectomy.
The patient was a 56-year-old male with vague upper abdominal pain. Investigations revealed a 3-cm x 3-cm mass arising from the duodenal mucosa with no evidence of extraserosal spread. Histopathology documented an adenomatous polyp with high-grade dysplasia, so a laparoscopic antrectomy was performed.
The patient had an uneventful postoperative period, requiring only 2 doses of parenteral analgesics. He was discharged on the seventh postoperative day. The final histopathological findings were consistent with benign hamartoma. No recurrence has been reported after 14 months of follow-up with endoscopy.
Many procedures have been described for polyps, such as endoscopic excision, duodenectomy, pancreatoduodenectomy, and laparoscopic polyp excision. In our patient, the decision to perform duodenectomy was based on the preoperative findings of a sessile tubulovillous adenomatous polyp with high-grade dysplasia. Histologically, the 2 entities can be identical, especially with the small tissue volume obtained from endoscopic biopsy.
Given these observations, antrectomy was probably ideal, because endoscopic excision would have been inadequate and even dangerous while pancreatoduodenectomy would have been too radical.
Duodenal polyp; Tubulovillous adenoma; Laparoscopic antrectomy; hamartoma
Benign triton tumor (BTT) or neuromuscular hamartoma is an uncommon tumor composed of mature neural and well-differentiated striated muscular elements. Its development is exceptionally rare in the adult and head region. This report describes a case of adulthood BTT that occurred in the orbit. The patient was a 53-year-old woman who presented with right periorbital swelling and pain in eyeball over 2 months. Magnetic resonance imaging revealed a well-enhancing mass surrounding optic nerve and ocular muscles in the right retrobulbar area. The tumor was subtotally removed via transcranial approach. Its pathological diagnosis was confirmed to be a neuromuscular hamartoma. She developed diplopia postoperatively. Adulthood BTT should be considered in the differential diagnosis of head and neck tumors. It is also important to make adequate therapeutic strategy to avoid postoperative neural dysfunction.
Adult; Benign triton tumor; Head; Neuromuscular hamartoma; Surgery
Hepatic mesenchymal hamartoma (HMH) is a hamartomatous growth of the mesenchymal tissue in the liver, which is of an uncertain aetiology. It is a space-occupying lesion that can potentially compress the adjacent organs, resulting in various complications, which include death. Hepatic mesenchymal hamartoma is characterized by the variable proliferation of the myxomatous mesenchyme and the malformed bile ducts. The differential diagnosis includes other paediatric hepatic masses. The diagnosis is typically made during infancy, and a complete resection is invariably curative. Here, we are reporting a case of HMH in a one year old female child who presented with abdominal distension. A clinical diagnosis of malignancy was made, based on the symptoms and imaging studies and a final diagnosis was made only after a histopathological examination. The lesion was potentially benign, with very rare chances of recurrence. This case has been presented due to its rarity.
Hepatoblastoma; Solid and cystic masses in the liver; Mesenchymal hamartoma; Bile ducts
Hamartoma of the breast is an uncommon lesion. Although it can possess characteristic radiological features, the pathological appearance is not distinctive. Hamartoma is generally considered benign, but four cases have been reported with ductal and lobular carcinoma arising in hamartomas. This report describes further cases of hamartoma from which ductal carcinoma in situ arose, with one showing early invasion. In both cases, the tumours were within the hamartomas and were adequately excised during lumpectomies of the hamartomas, and the patients were well afterwards. This report emphasises the importance of adequate sampling of mammary hamartoma.
hamartoma; ductal carcinoma in situ; fibroadenoma
Germ-cell tumors (GCT) are a histologically and biologically diverse group of neoplasms which primarily occur in the gonads but also develop at different extragonadal sites in the midline of the body. The head and neck region including the upper respiratory tract is a very rare location for such tumors in both children and adults, which can cause diagnostic and therapeutic difficulties.
We describe here two new cases of multilineage tumors including sinonasal teratocarcinosarcoma [SNTCS], and congenital oronasopharyngeal teratoma (epignathus) and compare their features with those of a new case of a rare salivary gland anlage tumor [SGAT], an entity for which the pathogenesis is unclear (i.e. hamartoma versus neoplasm). We correlate their presenting clinico-pathological features and compare histologic and cytogenetic features in an attempt to elucidate their pathogenesis and biologic potentials.
Results and discussion
Cytogenetic analysis revealed chromosomal abnormalities only in the case of SNTCS that showed trisomy 12 and 1p deletion. Both cytogenetic abnormalities are characteristically present in malignant germ cell tumors providing for the first time evidence that this rare tumor type indeed might represent a variant of a germ cell neoplasm. The SGAT and epignathus carried no such cytogenetic abnormalities, in keeping with their limited and benign biologic potential.
The comparison of these three cases should serve to emphasize the diversity of multilineage tumors (hamartomas and GCT) of the upper respiratory tract in regards to their biology, age of presentation and clinical outcomes. Malignant tumors of germ cell origins are more likely to affect adults with insidious symptom development, while benign tumors can nevertheless cause dramatic clinical symptoms which, under certain circumstances, can be fatal.
Brunner’s gland hamartoma is a rare benign tumour of the duodenum. It was first described by Cruveilhier in 1835. Presently around 200 cases have been reported in literature. No sex predilection is seen. Patients usually present in the fifth to sixth decades of life. They may be clinically silent or may present with variable symptoms and occasionally obstructive symptoms and chronic pancreatitis. Endoscopic presentation can be nodular, polypoid or diffuse glandular proliferation with thickening of duodenal wall and hence can be misdiagnosed as malignancy. We describe a case of duodenal tumor reported outside (on biopsy) as well differentiated adenocarcinoma which out as Brunner gland hamartoma upon complete resection. Brunner gland hamartoma may sometimes have a very unusual presentation. Extensive pre-operative evaluation is necessity to avoid radical surgical procedure.
Brunner glands; Hamartoma; Duodenum; Malignancy